Serum Iron Levels and Iron Deficiency Anemia in Children

ABDULRAHMAN BASHIRE CHILDREN HOSPITAL -- BENGHAZI 1
1. The serum iron is often raised in the following condition:-

A. Anaemia of chronic disorders.
B. Beta thalassemia.
C. Iron deficiency anaemia.
D. Sideroblastic anaemia.
E. Congenital spherocytosis
Ans: -BD
2. All of the following statements are true Except: -
A. Approximately 10% of RBCs are normally removed each day and replaced by the marrow to
maintain the RBC count
B. When the capacity of the heme-binding proteins in the plasma is exceeded, free hemoglobin
appears in the plasma
C. The most common cause of aplastic crisis is parvovirus B19 infection
D. The marrow can increase its output of RBCs two- to threefold acutely
E. The usual marrow response to a chronic hemolytic anemia is reflected by a reticulocyte index of
3-4
Ans:- A
Normal RBC survival time is 110120 days (half-life, 5560 days), and approximately 1% of RBCs (the
senescent ones) are removed each day and replaced by the marrow to maintain the RBC count.
3. All of the following statements are true Except:
A. The anatomic site of hematopoiesis changes during gestation and the population of cells
generated at those sites are distinct
B. Few neutrophils are found in the fetal circulation until the third trimester
C. Thrombopoietin is the physiologic regulator of platelet production but does not act as a potent
stimulator of all stages of megakaryocyte growth and development
D. Erythropoiesis in utero is controlled by erythroid growth factors produced solely by the fetus
E. Some HbA can be detected in even the smallest embryos
Ans:- C
Thrombopoietin (TPO) is the physiologic regulator of platelet production and acts as a potent stimulator of
all stages of megakaryocyte growth and development.
4. Regarding platelets:
A. They have a life span of approximately 10 days
B. Platelet production is controlled by specific cytokines
C. Platelets should be stored between 2 and 6 degrees C
D. Platelets can be stored for a maximum of 48 hours
E. A pool of platelets transfused can produce an increment of 50 000 / ml if platelet consumption is
not an issue
Ans:-AB
Platelets are produced from megakaryocytes, which are regulated by thrombopoietin, a specific cytokine.
They have a lifespan of 10-12 days thereafter being destroyed in the spleen.
Platelet concentrates should be stored at around 20 degrees C and the pH kept between 6.2 and 7.8 - these
conditions reduce the risk of a change in morphology of the platelets. They should also be continuously
agitated to encourage gas exchange.
One pool of platelets gives on average, an increment of 10 000 / ml.
5. All of the following statements regarding iron deficiency are true Except::-
A. Because absorption of dietary iron is assumed to be about 10%, a diet containing 80-100 mg of
iron daily is necessary for optimal nutrition
B. Intense exercise conditioning may result in iron depletion in adolescent girls
C. Iron deficiency may have effects on neurologic and intellectual function
D. The level of serum ferritin provides a relatively accurate estimate of body iron stores in the
absence of inflammatory disease
E. The red cell distribution width (RDW) is elevated in iron deficiency but not in and
thalassemia trait
Ans:-A
6. The following is true of iron deficiency anaemia:-
A. Is the commonest cause of microcytic hypochromic anaemia in infancy
B. Serum transferrin is the most reliable indicator other than BM aspiration, of total iron stores.
C. Is commonly present from 2 months of age upward in term infant.
D. The ratio of MCV to the red blood cell count is useful in differentiation from thalassemia.
E. Decreased T cell function and cell mediated immunity is reported.
Ans:ADE
Low birthweight and unusual perinatal hemorrhage are associated with decreases in neonatal hemoglobin
mass and stores of iron. As the high hemoglobin concentration of the newborn infant falls during the first 2
3 mo of life, considerable iron is reclaimed and stored. These reclaimed stores usually are sufficient for
blood formation in the first 69 mo of life in term infants. In low-birthweight infants or those with perinatal
blood loss, stored iron may be depleted earlier, and dietary sources become of paramount importance.
7. A 2 year old Pakistani boy has a haemoglobin of 8g/dl and an MCV of 65. The following tests are
essential:
A. Serum ferritin
B. Serum B12
C. Serum folate
D. Faecal occult blood
E. Haemoglobin electrophoresis
Ans:-ADE
Comments:
In progressive iron deficiency, a sequence of biochemical and haematologic events occurs. First, the tissue
iron stores represented by bone marrow haemosiderin disappear. The level of serum ferritin, an iron-storage
protein, provides a relatively accurate estimate of body iron stores in the absence of inflammatory disease.
Normal ranges are age dependent, and decreased levels accompany iron deficiency. Next, there is a decrease
in serum iron (also age dependent), the iron-binding capacity of the serum (serum transferrin) increases, and
the percent saturation (transferrin saturation) falls below normal (also varies with age). When the availability
of iron becomes rate limiting for haemoglobin synthesis, a moderate accumulation of heme precursors, free
erythrocyte protoporphyrins (FEP), results. As the deficiency progresses, the red blood cells (RBCs) become
smaller than normal and their haemoglobin content decreases. The morphologic characteristics of RBCs are
best quantified by the determination of mean corpuscular haemoglobin (MCH) and mean corpuscular
volume (MCV). Developmental changes in MCV require the use of age-related standards for diagnosis of
microcytosis . With increasing deficiency the RBCs become deformed and misshapen and present
characteristic microcytosis, hypochromia, poikilocytosis and increased red cell distribution width (RDW).
Iron deficiency is much commoner in more economically deprived communities, with up to 50% being
affected in some inner city areas.Detailed investigation is therefore unnecessary in this child, unless he fails
to respond to a trial of iron therapy.
8. Iron deficiency anaemia;-
A. Is characterized by low serum iron and low TIBC
B. Is associated with pica
C. Is prevented by the early introduction of cow's milk
D. Never require treatment under the age of 6 months
E. In childhood is associated with chronic blood loss in most cases
Ans:- B
9. The following cause a microcytic anaemia:
A. Ulcerative colitis.
B. Pernicious anaemia.
C. Methotrexate therapy.
D. Folate deficiency.
E. -Thalassaemia
Ans:-AE
10. Iron deficiency anaemia
A. May be prevented by the promotion of milk intake.
B. Contribute to more significant anaemia in infants who are breast rather than formula fed for
the first 6 months.
C. Is associated with higher incidence of early learning problems.
D. Is confirmed by demonstrating elevation of serum ferritin conc.
E. Increased susceptibility to infection.
Ans:-CE
Iron is absorbed 2 to 3 times more efficiently from human milk than from cow's milk. Breast-fed infants
may, therefore, require less iron from other foods.
11. The following are predisposing factors for iron deficiency anaemia:
A. Drinking unmodified cow's milk
B. Prematurity
C. Infant of diabetic mother
D. Intake of bottle milk
E. Excessive tea drinking
Ans:-ABE
Comments:
The predisposing factors/causes include:
Inadequate dietary intake of iron.
Drinking unmodified cow's milk (doorstep milk).
Prematurity and low birth weight.
Food stuffs and beverages which reduce iron availability including tea.
12. Serum ferritin:
A. Is raised in acute rheumatoid arthritis.
B. Stores 95% of the body's iron.
C. Is a useful measurement of iron storage in the body.
D. Should be measured in all cases of suspected iron deficiency.
E. Is increased in hepatoma.
Ans:-AC
Comments:
Iron is an essential component in the structure of haemoglobin and myoglobin for oxygen and carbon
dioxide transport. It is also found in oxidative enzymes, cytochrome C and catalase. It is absorbed in the
ferrous form according to body need, aided by gastric juice and Vitamin C, and hindered by fibre, phytic
acid, and steatorrhoea (about 90% of intake is excreted in the stool). It is transported in the plasma in the
ferric state bound to transferrin, and is stored in the liver, spleen, bone marrow and kidney as ferritin and
haemosiderin. It is conserved and reused with minimal losses in the urine and sweat. In progressive iron
deficiency, a sequence of biochemical and haematological events occurs:
First: Tissue iron stores (bone marrow haemosiderin) disappear, and serum ferritin drops. Ferritin is a
relatively accurate estimate of body iron stores in the absence of inflammatory disease.
Next: Serum iron drops, and TIBC increases, and free erythrocyte protoporphyrins begin to accumulate.
Later: Red cells become smaller, and hypochromic with a drop in MCH and MCV. There may be
poikilocytosis and increased red cell distribution width. Reticulocyte count may be normal or
elevated, with nucleated red cells seen on the peripheral film. There may be thrombocytosis. The
bone marrow is hypercellular with erythroid hyperplasia. Following iron therapy there is replacement of
intracellular iron enzymes and a subjective improvement within 24 hours. Within 48 hours there is a bone
marrow response, with reticulocytosis evident from 2 days, and peaking at about 7 days. The haemoglobin
level begins to increase from day 4 to day 30, and 3 months are required for complete repletion of iron
stores.
In most cases of suspected iron deficiency a low Hb plus microcytosis with response to iron therapy
obviates confirms the diagnosis, and there is no need to measure ferritin.
13. Regarding body iron stores:-
A. serum ferritin is a poor guide to iron stores
B. a healthy young man will have more iron stored as ferritin than in the circulating red cell mass
C. iron is the stimulant for ferritin production
D. in iron deficiency stores of haemosiderin are mobilized before ferritin
E. if stained with nitroprusside stain both haemosiderin and transferrin have same colour
Ans:- C
14. Regarding iron deficiency anaemia:
A. The commonest cause in children is chronic blood loss.
B. It does not affect school progress.
C. It commonly co-exists with thalassaemia.
D. Cow's milk is a major source of iron for children.
E. Lead poisoning is commonly associated with iron deficiency.
Ans:- E
Comments:
Iron is absorbed in the proximal small intestine, mediated partly by the duodenal protein mobilferrin. About
10% of dietary iron is absorbed, and iron is absorbed 2-3 times more efficiently from human milk than from
modified cow's milk. During the first years of life, because relatively small quantities of iron-rich foods are
taken, it is often difficult to attain sufficiency iron. The diet should include foods such as infant
cereals or formulae that have been fortified with iron. Breast fed infants should receive iron supplements
from 4 months of age. At best, the infant is in a precarious situation with respect to iron. Should the diet
become inadequate, or external blood loss occur, anaemia ensues rapidly. In children with microcytic
anaemia who fail to respond to iron, thalassaemia should be considered. In this country there is an increased
incidence in those from the Mediterranean and those from the Indian sub-continent. Because many such
children are socioeconomically disadvantaged, there may be an associated iron deficiency anaemia. Lead
poisoning in this country is usually associated with eating lead paint. Since pica is associated with iron
deficiency, the two often co-exist.
15. In iron deficient anaemia
A. Hypochromia, microcytosis, and reduced red cell count are typically found.
B. Pencil cells may be seen in the blood film
C. Koilonychia and glossitis are common complications
D. Free erythrocyte protoporphyrin levels are elevated
E. Ingestion of cows milk before the age of 1 year is a recognized cause
Ans;-ABDE
16. regarding anaemia in childhood:-
A. Iron deficiency anaemia is the most common cause.
B. Mucosal pallor is useful clinical sign.
C. Macrocytic blood film indicates iron deficiency anaemia.
D. Blood transfusions are standard treatment.
E. Occur in less than 5% of population.
Ans :-AB
Iron deficiency is indeed common; in some population has been quoted as high as 30%.
17. The commonest presentation in child with iron deficiency anemia:
A. anaemia
B. Palpitation
C. usually asymptomatic
D. poor concentration
E. heart failure
Ans :-CD
18. Concerning sideroblastic anaemia:
A. Ring sideroblasts are seen in the peripheral blood film.
B. Pancreatic exocrine dysfunction may occur.
C. It is associated with a low serum iron.
D. It may occur with anti-tuberculous therapy.
E. Some cases may improve with pyridoxine.
Ans:-BE
Comments:
The sideroblastic anaemias are a heterogeneous group of hypochromic, microcytic anaemias, probably due
to abnormalities of heme metabolism. Serum iron levels are raised, and ring sideroblasts are seen in the bone
marrow (nucleated red cells with perinuclear haemosiderin granules that represent iron-laden mitochondria).
Sub-types include:
Pearson Syndrome: associated exocrine pancreatic dysfunction due to deletions in mitochondrial DNA.
X-linked recessive: associated with splenomegaly. This may be pyridoxine sensitive or pyridoxine
refractory.
Acquired: various inflammatory and malignant processes, alcoholism.
19. The following are valid association in children with hypochromic anaemia:-
A. Normal serum ferritin &an elevated HbA
2
point to beta thalassemia minor
B. Low ferritin & normal HbA
2
exclude beta thalassemia minor.
C. Low ferritin point to iron deficiency.
D. Raised ferritin & normal hemoglobin electrophoresis point to anaemia of chronic
inflammatory disorder.
E. Dimorphic blood film is consistent with recent blood transfusion.
Ans:-ACDE
20. Which of the following statement regarding iron in infancy is false :-
A. About 4% of iron in fortified cow milk formula is absorbed by the infants
B. About 10% of iron in unfortified cow milk formula is absorbed by the infants
C. About 15% of iron in breast milk is absorbed by the infants
D. Absorption of iron by the infant is generally greater than in adults
E. Cow's milk and human milk have approximately the same iron content
Ans:- C
21. Hypochromic microcytic anaemia is seen in:
A. Alpha thalassemia
B. Sickle cell anemia
C. Autoimmune hemolytic anemia
D. Folate deficiency
E. Imerslund syndrome
Ans:- A
Comment:-
Hypochromic microcytic anemia is usually associated with:
a) Fe deficiency
b) Sideroblastic anemia:
- Primary X-linked recessive
- Secondary alcohol
- Malignancy
- Inflammation
In sideroblastic anemia there is Fe, but accumulation in the RBC mitochondria results in stippling on the
peripheral smear and ring sideroblast formation in the bone marrow.
Treatment is with pyridoxine (vitamin B6).
c) Lead poisoning
d) Beta-thalassemia (major or intermedia)
e) Alpha-thalassemia
f) Anemia of chronic disease
g) Copper deficiency
Sickle cell disease does not produce a microcytic anemia unless associated with thalassemia as in HbSb0.
To differentiate between Fe deficiency and beta-thalassemia use:
'Discriminant function'(DF) = MCV (fl) - RBC (x10
9
/l) - (5 x Hb [g/dl]) - 3.4
If DF is +ve = Fe deficiency
If DF is -ve = beta-thalassemia trait
Beware; this is no use in hemodilution states (i.e. post hemorrhage or in pregnancy).
Megaloblastic anemia on the other hand is usually due to:
a) Folate deficiency
b) Vitamin B12 deficiency
c) Orotic aciduria
d) Pyruvate kinase deficiency
To discriminate between folate and B12 deficiency - think:
-Decreased RBC folate
-Hyper-segmented neutrophils
-LDH
All suggest folate deficiency.
Imerslund syndrome is characterized by:
a) Chronic mucocutaneous candidiasis
b) Decreased PTH
c) Proteinuria
d) Decreased vitamin B12 absorption.
e) congenital autosomal recessive disorder which presents in the first 2 years of life as
megaloblastic anemia and failure to thrive.
22. Regarding anaemia in childhood:-
A. Hemoglobin concentration of 11g/dl at the age of 3 months merits investigation.
B. Iron supplements should be given to all breast feeding infants for the first 6 months of life.
C. Serum iron is characteristically raised and iron binding capacity reduced in chronic hemolytic
anemia.
D. The majority of cases of iron deficiency anaemia in the first 2 year of childhood are
nutritional in origin.
E. In the treatment of iron deficiency anaemia, parenteral iron therapy raises the hemoglobin
faster than oral iron.
Ans:-CD
23. In the anaemia of chronic infection:-
A. The hemoglobin is usually <8g/dl
B. The TIBC is raised
C. The anaemia is usually normocytic
D. The serum iron is usually reduced
E. Iron supplements are contraindication
Ans:-CD
24. Anaemia of acute infection and chronic disease is associated with all the following except:-
A. Shortened red cell survival
B. Impaired iron utilization
C. Iron stores are usually reduced
D. Impaired erythropoietin and bone marrow response
E. Usually mild anaemia with normocytic or microcytic red cells in acute infection
Ans:- C
25. Regarding blood indices:
A. The haemoglobin concentration of a 3 month old boy is higher than a 13 year old boy.
B. The mean corpuscular haemoglobin is low in megaloblastic anaemias.
C. The mean corpuscular haemoglobin concentration is low in iron deficiency anaemias.
D. The reticulocyte count increases with each year of life.
E. Reticulocytes are similar in size to mature red blood cells.
Ans:- C
Comments:
The haemoglobin at birth ranges from 13.7 - 21.1g/dl, falling to 13.0 - 20g/dl at 2 weeks of age, and to
between 9.5 and 14.5g/dl at 3 months. From there it rises gradually, with normal ranges between 6 months
and 6 years being 10.5 - 14.0g/dl, and between 7 and 12 years being 11.0 - 16.0g/dl. Adult normal ranges for
females are12.0 - 16.0g/dl, and for males 14.0 - 18.0g/dl. The mean cell haemoglobin (MCH) is the
haemoglobin divided by the red cell count. In health, this is usually between 27 and 32pg. Any disorder
which reduces red cell size reduces the amount of haemoglobin in the cell, and lowers the MCH. Likewise,
disorders increasing cell size raise the value. Means corpuscular haemoglobin concentration (MCHC) equals
the haemoglobin divided by the haematocrit. The normal range is 30 - 35g/dl. The MCHC is thus not the
number of grams of haemoglobin in 1dl of blood, but in 1dl of pure red cells without plasma. A low MCHC
is usually due to iron deficiency. The reticulocyte count in cord blood is 5%, paralleling the change from Hb
F to Hb A. From 2 weeks of age it drops to 1%, and remains at this level until adulthood. Once menses start,
females have a slightly higher reticulocyte count of around 1.6%. Reticulocytes are slightly larger than
mature red cells because of the little remaining nuclear material.
26. Concerning vitamin B12:-
A. Deficiency can cause ataxia
B. Pernicious anaemia commonly occur following terminal ileal resection
C. In deficiency MCV is usually normal
D. Deficiency usually occur in untreated celiac disease
E. Extrinsic factor promote absorption
Ans:- A
27. Juvenile pernicious anaemia is characterized by all of the following except:-
A. Gastric atrophy
B. Concurrent endocrinopathy
C. Selective IgA deficiency
D. Autosomal recessive inheritance pattern
E. Chronic candidiasis
Ans;-
28. Abnormality associated with extravascular hemolysis include:-
A. Increase in plasma hemoglobin
B. Decrease in serum haptoglobin
C. Increase in urinary hemosiderine
D. Increase in serum unconjugated bilirubin
E. None of the above
Ans:- D
29. With respect to hereditary hemolytic anaemia:-
A. Hereditary spherocytosis is the commonest hereditary hemolytic anaemia in Northern Europe.
B. Hereditary spherocytosis is confirmed by the presence of spherocytes on the blood smear and by
chromosome fragility test.
C. G6PD deficiency & pyruvate kinase deficiency are inherited in an autosomal recessive manner.
D. G6PD deficiency is confirmed by assaying the red cell G6PD enzymes.
E. The Embden-Meyhof pathway is unable to function in the red cell that is deficient in G6PD.
Ans:-AD
30. Vitamine E deficiency is associated with all of the following except:-
A. Greater prevalence in small premature infants.
B. Manifestation as anaemia with an elevated reticulocyte count.
C. Thrombocytosis.
D. Physical manifestation characteristic of this deficiency are edema of the legs , labia,and eyelids
in infants.
E. Result in an elevated alpha tocopherol level.
Ans:- E
31. All of the following statements regarding hereditary spherocytosis are true except :
A. Individuals with hereditary spherocytosis may be asymptomatic without anemia
B. The newborn with hereditary spherocytosis may present with anemia and .hperbilirubinemia
severe enough to require phototherapy and exchange transfusion
C. Isoimmune hemolytic anemia due to ABO incompatibility may mimic hereditary spherocytosis
D. Thermal injury can cause spherocytosis
E. Splenectomy does not eliminate most of the hemolysis of hereditary spherocytosis
Ans:- E
Because the spherocytes in hereditary spherocytosis are destroyed almost exclusively in the spleen,
splenectomy eliminates most of the hemolysis associated with this disorder. After splenectomy, osmotic
fragility often improves because of diminished splenic conditioning and less RBC membrane loss, and the
anemia, reticulocytosis, and hyperbilirubinemia resolve.
32. Feature of hereditary spherocytosis
A. Splenomegaly
B. Conjugated hyperbilirubinemia
C. Gallstones
D. Hemolytic crises following fava bean ingestion
E. X linked inheritance
Ans:-AC
33. Hereditary spherocytosis:-
A. Is usually inherited in sex- linked recessive fashion.
B. May necessitate exchange transfusion in the neonatal period.
C. Should routinely be treated by Splenectomy in infancy.
D. Can't be diagnosed in asymptomatic patient.
E. May be complicated by gall stone.
Ans:-BE
34. Concerning hereditary spherocytosis:
A. It is inherited as an autosomal dominant disorder.
B. The red cells have increased osmotic resistance.
C. Sufferers are prone to aplastic crises secondary to parvovirus infection.
D. It is associated with macrocytosis.
E. The cell shape returns to normal following a splenectomy.
Ans:-AC
Comments:
Hereditary spherocytosis (HS) is a common cause of haemolysis and haemolytic anaemia, with a prevalence
of 1:5000 in Northern Europeans. Patients may be asymptomatic without anaemia and minimal haemolysis,
or have severe haemolysis. It is autosomal dominant, though it is occasionally transmitted as AR. 25% of
patients have no family history. The abnormality is in spectrin, a major component of the cytoskeleton. This
results in loss of membrane without a proportionate loss of volume, so the red cells end up as small spheres
rather than biconcave discs. There is an associated increase in cation permeability and transport, ATP
utilisation, and glycolytic metabolism. The cells have decreased deformability impairing splenic passage, so
the cells are prematurely destroyed. In addition to haemolysis, hypoplastic crises may be associated with
parvovirus infection. This may result in profoun anaemia with high output heart failure, hypoxia,
cardiovascular collapse and death. In HS the haemoglobin level is usually 6 g/dl, and the reticulocyte count
is elevated to 6-20%. The MCV is normal, but the MCHC is increased. Spherocytes have a smaller diameter
than normal cells. The diagnosis is confirmed by an osmotic fragility test. Splenectomy eliminates most of
the haemolysis associated with the disorder, but carries its own risks. In mild cases, folic acid 1mg/day is
administered to prevent secondary folate deficiency. For those with severe anaemia, and/or hypoplastic or
aplastic crises, splenectomy is recommended after the age of 5-6 years. Vaccines for pneumococcus,
meningococcus and haemophilus influenzae should be given prior to splenectomy, and prophylactic
penicillin continued for life.
35. In hereditary spherocytosis ,all of the following are characteristic except:-
A. It is autosomal dominant trait
B. There is loss of red cell membrane surface without reduction of cell volume ,necessitating
spherical shape in order to accommodate it's content
C. Spherocytes are more likely to rupture than normal cells when suspended in hypotonic solution
D. The MCV is increased & the MCHC is decreased
E. Clinical severity tend to be relatively consistent within families although it varies widely from
family to family
Ans:-D
36. Hereditary spherocytosis :-
A. Is inherited as an autosomal dominant condition.
B. May be confirmed by paper electrophoresis of whole blood.
C. May cause sever jaundice in the newborn period.
D. May be complicated by abrupt exacerbation of anaemia due to transient erythroid hyperplasia
E. Is an indication for Splenectomy in the first 3 year of life.
Ans:-ACD
37. Concerning hereditary spherocytosis:-
A. Presence of spherocytes in the neonatal period is diagnostic of this condition .
B. Inheritance is as an autosomal dominant .
C. The direct coomb's test is positive in > 50% of cases.
D. The condition may present as jaundice in the neonatal period.
E. Splenectomy reverses the tendency to form spherocytes.
Ans:-BD
38. In child presenting with acute hemolysis due to an inherited hemolytic anaemia:-
A. Anaemia is present only after complete failure of haemtopoiesis.
B. The plasma conjugated bilirubin level is elevated.
C. Reticulocyte count will be less than 1%.
D. Plasma haptoglobin will be elevated.
E. There is excess urinary urobilinogen.
Ans;- E
39. Characteristic of G6PD deficiency in patient include all of the following except:-
A. Sex linked recessive patter of inheritance
B. Persistence of low grade anaemia in patient
C. The most common clinical manifestation is episodic acute hemolysis usually follow infection or
drug ingestion
D. In black G6PD activity is near normal in reticulocytes and young erythrocytes
E. Heinz bodies may be present only early in hemolytic episodes
Ans:- B
40. Glucose-6-phosphate dehydrogenase deficiency:
A. Is inherited as a sex-linked condition
B. is not clinically manifest in girls
C. Causes drug-induced haemolysis
D. Is an indication for Splenectomy
E. Is more pronounced in mature red blood cells
Ans:-ACE
Comments:
G6PDH deficiency is x linked recessive. It can manifest in females if there are 2 copies of the abnormal
gene. Drugs which cause haemolysis include aspirin, antimalarials, antibacterials and sulphonamides.
Splenectomy is not helpful in the management of this condition. Young red blood cells have near
normal enzymatic capacity.
41. All of the following statements regarding G6PD deficiency are true except :-
A. Symptoms usually develop in patients with G6PD deficiency 24-48 hr after ingesting a
substance with oxidative properties
B. Infection may result in hemolysis in patients with G6PD deficiency
C. A pregnant woman who ingests oxidative drugs may cause hemolytic anemia in a fetus with
G6PD deficiency
D. Enzyme activity in affected persons is 10% of normal or less
E. The usual dose of aspirin causes clinically relevant hemolysis in the A variety of G6PD
deficiency
Ans:- E
The usual doses of aspirin and trimethoprim sulfamethoxazole do not cause clinically relevant
hemolysis in the A- variety. However, aspirin administered in doses used for acute rheumatic fever (60
100 mg/kg/24?hr) may produce a severe hemolytic episode. When hemolysis has occurred, supportive
therapy may require blood transfusions, although recovery is the rule when the oxidant agent is
removed.
42. All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false
except:
A. Is more common in women than in men.
B. Is caused by mutations of the G6PD gene on chromosome 4.
C. Often leads to chronic haemolysis.
D. Requires treatment with desferrioxamine to prevent iron overload.
E. Is associated with haemolytic crises induced by sulfonamides.
Ans:- E
G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. The NADPH is then
used to reduce glutathione and hence in conjunction with glutathione reductase reduces cellular oxidative
damage.
G6PDH enzyme deficiency, caused by mutations in the X-linked (Xq28) G6PD gene, affects over half a
billion people worldwide. It is a balanced polymorphism associated with resistance to falciparum malaria in
heterozygous females. This evolutionary advantage outweighs the small negative effect of affected
hemizygous males. At least 400 G6PD variants have been identified. The various mutations lead to altered
expression of G6PD or an altered half-life of the enzyme.
There are a number of disease manifestations:
- patients may be asymptomatic
- patients may present with neonatal jaundice
- usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e.g.
certain foods - fava bean, chemicals - naphthalene, certain drugs - dapsone, primaquine, chloroquine, aspirin
(high doses), sulphonamides, nitrofurantoin, vitamin K and nalidixic acid.
Infections can also provoke haemolysis. Haemolytic crises vary in severity, rarely chronic haemolysis may
occur. If severe, G6PD deficiency can cause immunodeficiency by reducing the NADPH required for
activation of the NADPH oxidase enzyme (cf. CGD).
43. Chronic intravascular haemolysis is associated with:
A. Splenomegaly.
B. Haemosiderinuria.
C. Increased incidence of gallstones..
D. Reduced transferrin levels.
E. Increased serum haptoglobins.
Ans:-BC
Comments:
Haemolysis is defined as the premature destruction of red cells. If the rate of destruction exceeds the
capacity of the marrow to produce red cells then anaemia results. The normal red cell survival is 120 days,
and 1% of red cells are removed per day and replaced by the marrow. During haemolysis, the red cell
survival is shortened, and there is increased marrow activity (raised reticulocyte count). Marrow output can
increase 2-3 fold acutely, and 6-8 fold in long-standing cases. In chronic haemolytic anaemia, erythroid
hyperplasia may be so extensive that the medullary spaces may expand at the expense of cortical bones
(particularly skull and long bones). Intravascular haemolysis increases circulating haemoglobin which binds
to haptoglobin reducing the circulating levels of it. There is an increase in urine haemoglobin and
haemosiderin. Increased bilirubin production from heme results in increased faecal urobilinogin, which is
reabsorbed and excreted in the urine.
44. The following drugs should be avoided in all patients with glucose-6-phosphate dehydrogenase
deficiency:
A. Quinine
B. Nitrofurantoin
C. Sulphapyridine
D. Chloramphenicol
E. Chloral hydrate
Ans:- ABCD
Comments:
In glucose-6-phosphate-dehydrogenase deficiency (G-6-PD) subjects are susceptible to developing acute
haemolytic anaemia on taking a number of common drugs. Ingestion of fava beans may result in haemolysis
in severe cases, or when they are eaten raw. G-6-PD is genetically heterogeneous. The risk from drugs
therefore varies from patient to patient. There is no test available to identify potential risk in G-6-PD
deficiency. The risk of severity of haemolysis is almost always dose related. Drugs with a definite risk in
most G-6-PD deficient subjects include:
Sulphonamides and Dapsone.
Methylene blue
Nitrofurantoin
Primiquine
Quinilones (including Ciprafloxacin, Nalidixic acid)
45. The following are associated with a definite risk of hemolysis in most glucose-6 phosphate
dehydrogenase deficient subjects:
A. Primaquine
B. Methyllene blue
C. Nalidixic acid
D. Co-trimaxazole
E. Nitrofurantoin
Ans:- ABCDE
glucose-6 phosphate dehydrogenase reduces NADP to NADPH. NADPH is used by glutathione reductase
to reduce (oxidised) glutathion (toGSH). This replenishes the supply of reduced-SH groups in the
erythrocyte membrane protecting it from oxidant stress.
Deficiency of G6PD renders the red cell membrane susceptible to oxidant stress and haemolysis may result
following exposure to an oxidising agent of the environment (drugs, fava beans, infections, DKA).
G6PD also reduces NAD to NADH which is used by methaemoglobin reductase. Therefore G6PD
deficiency may also result in increased oxidation of Hb (metHb) with the apperarance of Heinz bodies on
the blood film. Drugs that cause hemolysis in G6PD deficiency are oxidizing agent. The BNF gives two lists
of drugs as follows:
(1)- Definite risk of hemolysis in most: 2)-Possible risk in some:
*dapson. *aspirin (high dose).
*methylene blue. *chloroquine.
*nitrofurantoin. *menadione (vit K).
*Primaquine. * probencid.
*quinolones *quinidine,.
*sulphonamides. *quinine
46. G6PD deficiency:-
A. Is inherited as sex-linked condition.
B. Is not clinically manifest in girls
C. Causes drug induced hemolysis.
D. Is an indication for Splenectomy.
E. Is more pronounced in mature RBC
Ans:-ACE
Most male show complete expression but female less so; American black the prevalence in male is 15% &
in female is 2%
47. Regarding glucose-6-phosphate dehydrogenase:
A. It is an important enzyme in the kreb cycle.
B. Deficiency is associated with an increased susceptibility to falciparum malaria infection.
C. Deficiency may be associated with chronic haemolytic anaemia.
D. Deficiency is inherited as an autosomal dominant.
E. Deficiency may be associated with primiquine-induced haemolysis.
Ans:- E
Comments:
Glucose-6-phosphase dehydrognease (G-6-PD) is one of the enzymes in the pentose phosphate pathway
(Hexose Monophosphate Shunt). The most important function of this pathway is to maintain glutathione in a
reduced state as protection against oxidation of the red cell. Deficiency is X-linked, and extremely common,
affecting more than 200 million people. Heterozygous females have increased resistance to falciparum
malaria, outweighing the small negative effect on hemizygous males. Major symptoms include:
Episodic or induced haemolytic anaemia: there is considerable variation in the defect among different
racial groups, with Mediterranean whites affected more severely than Africans. Haemolysis may precipitated
by:
1. Drugs: Antibacterials: sulfonamides, septrin, nalidixic acid, chloramphenicol, nitrofurantoin.
Antimalarials: primaquine, chloroquine, quinacrine. Others: Vitamin K, methylene blue, Aspirin.
2. Chemicals: Phenylhydrazine, benzene, naphthalene.
3. Illness: Diabetic ketoacidosis (DKA), hepatitis.
Chronic haemolytic anaemia: chronic non-spherocytic haemolytic anaemia is associated with profound
deficiency of G-6-PD. Splenectomy is of little value in these types.
48. Regarding autoimmune hemolytic anaemia (AIHA):-
A. The direct coombs test will be positive.
B. It is frequently due to an intercurrent viral infection in children.
C. Spherocytes are commonly seen on the blood smear.
D. AIHA is divided into warm & cold types depending on whether the patient is febrile or afebrile.
E. It is often associated with connective tissue disorders in the adolescent.
Ans:-ABCE
49. A 17 year old Nigerian presents with moderately enlarged spleen, haemoglobin of 7.2 g/dL, a red
cell count of 3.5 x 1012/L, and a reticulocyte count of 7%. The following statements are correct:
A. Recent chloramphenicol administration would be of diagnostic significance
B. If the Coombs' test was positive, paroxysmal nocturnal haemoglobinuria is possible
diagnosis
C. Homozygous thalassaemia is possible diagnosis
D. If Heinz bodies are present, red cell glucose 6-phosphate dehydrogenase deficiency is
likely diagnosis
E. homozygous sickle cell disease is likely diagnosis.
Ans:-C D
Comments:
b-thalassaemia major (homozygous) thalassaemia is possible diagnosis. Splenic atrophy from splenic
infarcts occurs in sickle cell disease. Chloramphenicol may cause aplastic anaemia - there will be no
reticulocyte response. Heinz bodies are oxidized denatured haemoglobin. During crisis of G6PD deficiency,
blood film also shows contracted and fragmented cells due to oxidant stress. PNH is not an autoimmune
haemolytic anaemia, hence Coombs test is negative. It is a rare acquired defect of red cell membrane making
it susceptible to lysis by complement.
50. Features which may be seen on the blood film of a patient with haemolysis include:
A. Polychromasia
B. Howell Jolly bodies
C. Elliptocytes
D. Target cells
E. Heinz bodies
Ans:-ABDE
Comments:
The following are common abnormalities of red cell morphology:
Polychromasia: younger cells have a bluish tinge (basophilia), and are larger than average, and may
contain residual nuclear material (reticulocytes). The presence of many basophilic forms in a blood film
produces a multicoloured effect known as polychromasia.
Microcytosis indicates small red cells, anisocytosis variation in size, and macrocytosis a large size.
Poikilocytosis indicates altered shape.
Spherocytes may indicate hereditary spherocytosis, and may also be found in acquired haemolytic
anaemias, while elliptocytes are found in hereditary elliptocytosis.
Schistocytes are fragments of red cells, as seen in DIC or haemolytic uraemic syndrome.
In splenic dysfunction: target cells and red cells containing nuclear fragments known as Howell Jolly
bodies are seen. Pappenheimer bodies are iron containing inclusions, and when seen together with Howell
Jolly bodies suggest previous splenectomy or reduced splenic function.
Heinz bodies: are denatured haemoglobin which result from deficiencies of enzymes of the penthose
phosphate pathway. This damages the red cell leading to haemolysis and removal of them by the spleen.
The following conditions are haemaglobinopathies:-
A. G6PD deficiency.
B. von Willebrand disease.
C. Spherocytosis.
D. Thalassaemia..
E. Sickle cell disease.
Ans:-DE
51. Homozygous beta thalassemia
A. Arise from alteration in the beta globin gene on chromosome 10 .
B. May present with FTT.
C. Is associated with elevated HbF in infancy & older children.
D. May require Desferoxamine treatment.
E. Doesn't responds to iron treatment despite microcytic indices.
Ans:-BCDE
52. Which is compatible with B-thalassemia major?
A. HbF 96% HbA
2
4%
B. Hb A
1
40%, Hb F 58%, Hb A
2
2% .
C. Hb A
1
85%, Hb F 10%, HbA
2
5%
Ans: A
53. In beta thalassaemia:-
A. Beta thalassaemia major presents at birth with profound anaemia
B. The reticulocyte count is low
C. Diagnosis can be made by Haemoglobin electrophoresis
D. Anaemic patients should receive regular iron supplementation
E. Splenectomy is contraindicated due to extramedullary haemopoiesis
Ans:- C
Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in
the synthesis of beta-globin chains. In the homozygous state, beta thalassemia (ie, thalassemia major)
causes severe transfusion-dependent anemia with high reticulocyte count.
The anaemia becomes manifest in late infancy. Diagnosis is by haemoglobin electrophoresis.
Increased iron deposition resulting from multiple life-long transfusions and enhanced iron absorption
results in secondary iron overload. This overload causes clinical problems similar to those observed with
primary hemachromatosis (eg, endocrine dysfunction, liver dysfunction, cardiac dysfunction)
o The physical findings are related to severe anemia, ineffective erythropoiesis, extramedullary
hematopoiesis, and iron overload resulting from transfusion and increased iron absorption.
o Skin may show pallor from anemia and jaundice from hyperbilirubinemia.
o The skull and other bones may be deformed secondary to erythroid hyperplasia with intramedullary
expansion and cortical bone thinning.
o Heart examination may reveal findings of cardiac failure and arrhythmia, related to either
severe anemia or iron overload.
o Abdominal examination may reveal changes in the liver, gall bladder, and spleen.
Hepatomegaly related to significant extramedullary hematopoiesis typically is observed.
Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to iron
overload; transfusion-associated viral hepatitis resulting in cirrhosis or portal hypertension also may be
seen. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolytic
state. Splenomegaly typically is observed as part of the extramedullary hematopoiesis or as a
hypertrophic response related to the extravascular hemolysis.
o Extremities may demonstrate skin ulceration.
o Iron overload also may cause endocrine dysfunction, especially affecting the pancreas,testes& thyroid.
54. In thalassemia:-
A. The diagnosis is made on blood film.
B. The anaemia is due to deficiency in synthesis of globin chain.
C. The anaemia is mainly due to hemolysis.
D. Splenectomy is the treatment of choice at diagnosis.
E. The serum iron level is normal at diagnosis.
Ans;- BE
55. The clinical picture of B-thalassemia major:-
A. Present at birth.
B. Is characterized by iron deficiency anaemia.
C. May initially present as FTT
D. Include hepatosplenomegaly.
E. Is confirmed if investigation reveals markedly raised HbF on electrophoresis.
Ans:- CDE
56. Beta thalassemia major:-
A. Is associated with relative excess of b globin molecules.
B. Result in blood film exhibiting microcytic hypochromic red cell.
C. Produce generalized osteoporosis.
D. Is commonly associated with reduced serum iron.
E. Result in delayed puberty.
Ans:- BCE
57. The following are characteristic finding in beta thalassemia :-
A. Hypochromic microcytic red cells
B. Low serum iron
C. Cardiomyopathy.
D. Presentation in the neonatal period.
E. Frontal bossing of the skull.
Ans:-ACE
58. Thalasemia major is associated with all of the following except:-
A. Defect in globin chain synthesis
B. In the newborn infant is clinically & hematologically normal
C. The determination of HbA2 is important in diagnosis
D. Onset of clinical symptoms & sign usually begin between 6-12 month of age
E. Peripheral blood show hypochromic and usually microcytic anaemia
Ans;- C
59. Which one of the following doesn't characterize heterozygote thalassemia ( minor / trait):-
A. In most patient anaemia & clinical symptoms are absent
B. Life expectancy is shortened
C. Varieties of this disorder are best distinguished by quantification of A2 & HbF
D. Peripheral blood examination reveal microcytosis ,target cells , & variable degree of
hypochromia
E. Severity depend on the degree of suppression of globin synthesis
Ans:- B
60. All of the following statements are true Except:
A. In patients who have Hb SS electrophoresis pattern and concomitant microcytosis, iron deficiency or
a combination of Hb S with or thalassemia must be considered a possible diagnosis
B. Febrile infants with sickle cell disease should be managed in the hospital
C. The iron in hemoglobin is normally in the ferric state, which is essential for oxygen transport
D. Children with homozygous thalassemia usually become symptomatic from progressive hemolytic
anemia during the second 6 mo of life if not treated
E. The thalassemia traits present as microcytic anemia, which can be mistaken for iron-deficiency
anemia
Ans:- C
61. A black West African girl aged 15 years is complaining of severe pain in both legs. The
haemoglobin is 8g/dl. Sickle cell anaemia is unlikely to be the cause of her symptoms if
A. she is jaundiced
B. she has haematuria
C. x-ray of the spine shows osteoporosis
D. menarche occured at 12 years
E. the urinary osmolality is 800mosm/kg (specific gravity approximately 1022)
Ans:- DE
Comments:
a+b-expected in sickle cell anaemia, c-x-ray usually shows fishmouth vertebrae (infarcts), d-growth +
development usually impaired, e-almost all are unable to produce concentrated urine
62. In the management of acute sickle cell painful crises:
A. Patients should receive continuous oxygen and monitoring of SaO2.
B. Patients should receive intravenous antibiotics.
C. Intravenous opiate analgesia should be titrated until adequate control of pain.
D. Blood transfusions should be given.
E. Intravenous hydroxyurea should be given.
Ans:-ABC
Comments:
Painful episodes can often be managed with oral Paracetamol -/+ codeine. Severe episodes require
hospitalisation with parenteral opiates. Anti-inflammatories may decrease or eliminate the need for
narcotics. Dehydration and/or acidosis should be rapidly corrected by intravenous fluids. Packed cells are
specifically indicated for acute splenic sequestration and aplastic crises. The latter may require splenectomy.
Intravenous antibiotics should be given to cover the possibility of haemophilus or pneumococcal infection.
Chemotherapy regimens that stimulate fetal haemoglobin synthesis have been employed with beneficial
effect on an experimental basis. These include hydroxyurea and hydroxybutyrate. These are given as
maintenance therapy.
63. The following are recognised features of sickle cell disease:
A. Dactylitis
B. Frontal bossing of the skull
C. Abdominal pain
D. Chronic leg ulceration
E. Anaemia from birth
Ans:- ACD
Comments:
Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature
destruction of brittle, poorly deformable erythrocytes. Other manifestations are due to ischaemia resulting
from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises. The
manifestations vary considerably with age.
Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle
dactylitis (hand-foot syndrome).
Pre-school: acute painful vaso-occlusion crises, affecting extremities.
School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any
individual patient. Episodes may be precipitated by intercurrent illness.
Late changes:
- infarction of bone marrow or bone.
- splenic infarction between 6 and 60 months contributing to autosplenectomy.
- pulmonary infarcts (acute chest syndrome).
- stroke caused by cerebrovascular occlusion -/+ hemiplegia.
- ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal function
and concentrating ability.
Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration.
Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by
pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child
ages, auto splenectomy reduces spleen size.
Cardiomegaly is invariably present in older children (sickle-related cardiomyopathy), secondary
haemosiderosis from increased iron absorption may damage liver, pancreas and heart, and there may be gall
stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in late adolescence.
64. A healthy, 39-week gestation male weighing 3.5 kg is born to a mother who has chronic anemia.
The infants physical examination findings are normal, and his hematocrit is 49% (0.49). The
mother asks you about the need for vitamins and iron for her newborn son.
Of the following, the BEST response is that term babies need to begin iron therapy at
A. birth
B. 2 weeks of age
C. 4 months of age
D. 9 months of age
E. no time
Preferred Response: C
Term newborns have accrued sufficient iron stores in the latter part of gestation to sustain them for 3 to 4
months after birth; this is true even when the mother has anemia. Although human milk contains lower
quantities of iron, its bioavailability is greater and, therefore, breastfed infants do not require replacement
therapy until 4 months of age. Preterm infants miss out on iron accretion in utero during the last trimester of
pregnancy and may require iron supplementation if they are taking full-volume enteral feedings as early as 2
to 4 weeks of age.
Iron supplementation is not required at birth except in the rare circumstance of congenital anemia. Iron
supplementation is required for normal hematopoiesis and brain growth and function, and if not provided by
6 months of age, characteristically leads to iron deficiency anemia.
65. You are called to the delivery room to evaluate an infant who has been delivered by spontaneous
vaginal delivery. The term infant weighs 3.6 kg and has some grunting respirations. You decide to
observe her in the newborn intensive care unit. One hour later, you are notified that the infant is
experiencing pronounced respiratory distress and oxygen saturations in the 70% range in the
right hand and in the 50% range in the right foot. There is poor perfusion. There are no murmurs,
but there is a pronounced precordial lift and a loud second heart sound.
Of the following, the MOST likely diagnosis for this infant is
A. congenital diaphragmatic hernia
B. congenital toxoplasmosis
C. hypoplastic left heart syndrome
D. persistence of the fetal circulation
E. tetralogy of Fallot
Preferred Response: D
Determining the cause of cyanosis and hypoxemia in the neonate is critically important, but it can be
difficult, especially during the first few minutes of the presentation. Diagnostic considerations in these
potentially critically ill infants may include persistence of the fetal circulation, with or without meconium
aspiration, and cyanotic congenital heart disease. The neonate described in the vignette clearly is
experiencing a disturbance in the process of oxygen delivery, and several clues suggest persistence of the
fetal circulation. Detecting this condition requires a clear understanding of the ductus arteriosus and its
function in the transitional circulation.
In the normal heart, the right atrium and right ventricle deliver desaturated blood to the organ of
oxygenation. In the fetus, this organ is the placenta, and its fetal blood supply is via the umbilical artery,
which arises from the fetal descending aorta. The ductus arteriosus provides a fetal shunting pathway,
allowing the right side of the heart to deliver desaturated blood to the placenta by shunting this blood away
from the high-resistance pulmonary arteries and into the descending aorta. This direction of flow is due in
part to the slightly higher fetal pulmonary vascular resistance compared with fetal systemic vascular
resistance because the lungs are filled with fluid and the placenta is a low-resistance circuit.
At birth, when the lungs expand with air and the placenta is removed from the circulation, pulmonary
vascular resistance falls and systemic vascular resistance increases. This leads to a reversal of flow across
the ductus arteriosus (from the system into the pulmonary circuit). Over
subsequent hours and days, the ductus arteriosus begins the process of spontaneous closure.
When the pulmonary vascular resistance exceeds the systemic vascular resistance, right-to-left shunting
across the ductus arteriosus persists as long as the ductus arteriosus is patent. Neonates who have severe
lung disease, including pneumonia, lung collapse, and pulmonary hypoplasia, may have pulmonary vascular
resistance that exceeds the systemic vascular resistance. When this occurs in the presence of a patent ductus
arteriosus, there is right-to-left shunting of blood in a pattern that is similar to the fetal circulation. Hence,
the term persistence of the fetal circulation, or perhaps better stated, persistent pulmonary hypertension
of the newborn. This condition leads to highly desaturated blood entering the descending aorta, often
evidenced by lower oxygen saturation in the lower body than in the upper body. When this process results
from pulmonary pathology, there is likely intrapulmonary ventilation-perfusion (V-Q) mismatch as well,
yielding the return of relatively desaturated blood to the left heart, which then delivers inadequately
oxygenated blood to the aorta.
Signs of an underlying pulmonary process may include grunting to maintain alveoli and small airway
patency, tachypnea, and ultimately respiratory distress and failure, as described for the infant in the vignette.
The oxygenation saturation pattern of the infant supports the diagnosis of persistence of the fetal circulation.
She has no murmur because she has no turbulent blood flow in her heart, and her precordial lift results from
the increased volume, pressure, and work load imposed on the right ventricle. Poor perfusion is caused by
failure (diminished ventricular function) of the right ventricle and its impact on the left ventricle.
Neonates who have diaphragmatic hernia often present with respiratory distress and a shift of the
precordium to the right chest because the gastrointestinal contents typically occupy the left thorax.
Infants who have toxoplasmosis may have signs and symptoms of systemic infection, but they are
unlikely to present solely with pulmonary pathology.
The hypoplastic left heart syndrome relies on right-to-left shunting at the ductus arteriosus to supply
systemic blood flow, but affected infants have normal lungs and low pulmonary vascular resistance, with
oxygen saturation typically in the 80% to 90% range.
Tetralogy of Fallot usually presents with the murmur of pulmonary stenosis, and when the ductus
arteriosus is present, blood flow is left to right because pulmonary vascular resistance and the lungs are
normal
66. In sickle cell disease:-
A. Dactylitis is common presentation in infancy.
B. Autosplenectomy has generally occurred by the age of 6 months.
C. Sickle lung is an indication for exchange transfusion.
D. Aplastic crises may arise as consequence of parvovirus infection.
E. Salmonella osteomyelitis occur.
Ans:-ACDE
Dactylitis, often referred to as hand-foot syndrome, is frequently the 1st manifestation of pain in children
with sickle cell anemia, occurring in 50% of children by 2 yr of age. Dactylitis often presents with
symmetric swelling of the hands and/or feet
Acute splenic sequestration is a life-threatening complication occurring primarily in infants, and may occur
as early as 5 wk of age. Approximately 30% of children with sickle cell anemia have significant splenic
sequestration episodes; Repeated episodes of splenic sequestration are common, occurring in approximately
50% of patients. The majority of recurrent events occur within 6 mo of the previous event.
67. Sickle cell disease :-
A. Is caused by a mutation on chromosome 16
B. Will give a positive sickle test result at birth
C. Is associated with strokes in 25% of patients by the age of 45 years
D. Is an indication for autologous haemopoietic stem cell transplant
E. Can be co-inherited with alpha or beta-thalassaemia
Ans:- CE
Sickle cell disease is an autosomal recessive condition caused by a single amino acid mutation in the Beta
globin gene on chromosome 11. The alpha globin genes are coded on chromosome 16. It can be co-inherited
with alpha or beta thalassaemia. Clinical effects do not become apparent until 6 to 12 months of age when
haemoglobin switching occurs and the amount of HbF (fetal haemoglobin) decreases and the amount of HbS
(sickle haemoglobin) increases. This is why the sickle test, which detects the presence of sickle
haemoglobin, is often not positive until 6-9 months of age and cannot be guaranteed until one year of age.
Clinical problems include strokes and these are common, occuring in 25% of patients with homozygous
sickle disease by the age of 45 years. Patients with severe clinical manifestations of sickle disease have been
treated with allogeneic bone marrow transplantation from an HLA-identical sibling. Autologous bone
marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit in
this condition.
68. Sickle cell disease :-
A. Is associated with increased the polymerization of deoxygenated Hb
B. Is recognized cause of prolonged neonatal jaundice.
C. Increased the susceptibility to infection with encapsulated bacteria.
D. Is cause of childhood stroke ( cerebrovascular disease ).
E. Is conventionally managed by regular blood transfusion.
Ans:-ACD
69. In sickle cell disease:-
A. There is abnormality of red cell membrane.
B. Symptoms result from tissue infarction.
C. There is some protection against Plasmodium falciparum malaria.
D. Sickling crises can be prevented by morphine.
E. Sickling risk can be reduced by exchange transfusion.
Ans:-BE
Individuals with sickle cell trait have lower levels of Plasmodium falciparum parasitemia, higher
hemoglobin counts, and less severe reinfections than individuals with homozygous Hb A.
70. Regarding sickle cell anaemia :-
A. Positive Sickling test is diagnostic of sickle cell disease
B. Roughly 5% of African are carriers of the sickle cell trait.
C. The heterozygous condition is usually asymptomatic.
D. Treatment with desferoxamine has significantly improved the life expectancy in sickle cell
disease.
E. There is increased predisposition to salmonella osteomyelitis.
Ans:-CE
the incidence of sickle cell trait is 710% of African-Americans (1 in 12. ). The amount of Hb S in
individuals with sickle cell trait is <50%.
71. The mother of a baby in the postnatal ward has an antenatal screening test for sickle haemoglobin.
This is positive deposite a normal Hb & MCV. The Hb-electrophoresis shows HbA 55% and Hbs
45%. Her partner has a microcytic anaemia, and Hb-electrophoresis shows an HbA band only
with HbA2 quantitation of 4.8%. Which diagnosis is impossible in the baby?
A. Sickle cell trait.
B. Haematologically normal.
C. B-thalassaemia trait.
D. Sickle-B-thalassaemia.
E. Sickle cell diseas(HbSS).
Ans:- E
The mother probably has a sickle cell trait,the father has no evidence of Hbs & has a laboratory & Hb-
electrophoretic profile compatible with B0thalassaemia trait.
The baby may therefore have :
*Normal Hb.
*Sickle cell trait.
*B-thalassaemia trait.
*Sickle-b-thalassaemia.
It is not possible for the baby to have sickle cell disease (HbSS).
72. Sickle cell anaemia:-
A. Is associated with increased risk of gallstone.
B. Is associated with increased risk of nocturnal enuresis
C. Can be diagnosed antenatally
D. Is an indication for prophylactic penicillin
E. Is associated with episodes of acute anaemia
Ans:-ABCDE
73. Complications of sickle cell disease include:
A. Pneumococcal infection in the first four weeks of life.
B. Stroke.
C. Dactylitis in the first four weeks of life.
D. Cholecystitis.
E. Haematuria.
Ans:-BDE
Dactylitis (swelling of the joints)is the most common presentation symptom of sickle cell disease. It usually
occurs between six to twelve months of age when haemoglobin production switches from the production of
gamma chains to the production of the abnormal beta chains, and may present to an orthopaedic surgeon.
This means that the amount of foetal haemoglobin (which is protective against sickle crisis)decreases and
the amount of sickle haemoglobin increases.
Pneumococcal infections (and infections with other encapsulated bacteria such as Haemophilus influenza B
and neisseria meningitides) are common in patients with sickle cell disease from the age of 2 to 3 years
when they have auto-infection of the spleen and are rendered hyposplenic. They should be protected against
these diseases with vaccination and prophylactic antibiotics.
Strokes are very common in patients with sickle cell disease occurring in 25% of patients with homozygous
sickle cell disease by the age of 45 years.
Cholecystitis occurs in patients with sickle cell disease as with a haemolytic anaemia because of the
production of pigment gallstones.
Haematuria occurs in sickle cell disease because of renal papillary necrosis. Interestingly it can also occur in
patients with sickle cell trait.
74. Which of the following statement is false regarding the pathophysiology of sickle cell disease:-
A. Infection is associated with increased anemia by suppression of RBC production
B. Conversion of RBC from normal biconcave discs to sickle form require the deoxygenation of
haemoglobin
C. Hypoxiemia and acidemia promote sickling by decreasing oxygen saturation of Hb
D. Dehydration doesn't promote sickling
E. Sickle cell increase whole blood viscosity producing local ischemia
Ans:-D
75. Which one of the following clinical manifestation isn't common to sickle cell disease:-
A. Vaso-occlusive crisis is more common than aplastic crisis
B. Hyperhemolytic crisis is often associated with red cell G6PD deficiency
C. Aplastic crisis is often associated with viral or bacterial infection
D. Salmonella sepsis is commonly associated with salmonella osteomyelitis
E. Hand-Foot syndrome may be initial manifestation of sickle cell disease
Ans:-D
76. The following are seen in sickle-cell anaemia
A. dactylitis
B. retardation of secondary sexual characteristics.
C. pathognomic fundal changes
D. cardiac signs simulating mitral stenosis
E. normal urinary concentrating ability only if the sickle-cell trait is present
Ans:-ABCD
Comments:
Dactylitis - inflammation of the fingers is a feature of SCD due to expansion of bone marrow. B -
aconsequnce of ill health with hypogonadotrophic hypogonadism. A so called black sunburst retinopathy is
pathognomonic of sickle cell disease. Retinal vein thrmbosis is also seen. Poor urinary concentration is a
feature of the disease but is unaffected with the trait.
77. Recognised features of sickle cell trait include:
A. Moderate anaemia
B. Increased risk of anaesthesia
C. Reduced renal concentrating ability in adolescence
D. Episodic haematuria
E. Splenomegaly
Ans:-BCD
Comments:
Heterozygous expression of the sickle haemoglobin gene (Hb AS) is usually associated with a totally benign
clinical course. The haematological findings are indistinguishable from normal. About 40% of their
haemoglobin consists of Hb S, and under normal circumstances this is insufficient to produce sickling.
Under severe hypoxic stress, vaso-occlusive complications may occur. This may occur under anaesthesia or
at high altitudes. This may result in splenic infarcts and other ischaemic sequelae. Decreased renal
concentrating ability is usually present in older children and adults, and occasional gross haematuria may
occur. The diagnosis is confirmed by haemoglobin electrophoresis and sickle testing.
78. A 10 year old West African boy presents with Hb 8g/dl and pains in his legs. Sickle cell disease is
unlikely if:
A. He is jaundiced.
B. He has gross splenomegaly.
C. Puberty began at 12 years.
D. His urine osmolality is 800mosmol/L, specific gravity 1022.
E. There is a mid systolic murmur.
Ans:-BCD
Comments:
Sickle cell anaemia is characterised by severe chronic haemolytic disease resulting from premature
destruction of brittle, poorly deformable erythrocytes. Other manifestations are due to ischaemia resulting
from vascular occlusion by masses of sickle cells. The clinical course is typically associated with crises.
The manifestations vary considerably with age.
Newborns: haemolytic anaemia from 2-4 months as fetal haemoglobin is replaced by Hb S, acute sickle
dactylitis (hand-foot syndrome).
Pre-school: acute painful vaso-occlusion crises, affecting extremities.
School children: painful crises affecting head, chest, abdomen, back, the site being typical for an any
individual patient. Episodes may be precipitated by intercurrent illness.
Late changes:
o infarction of bone marrow or bone.
o splenic infarction between 6 and 60 months contributing to autosplenectomy.
o pulmonary infarcts (acute chest syndrome).
o stroke caused by cerebrovascular occlusion -/+ hemiplegia.
o ischaemic damage to myocardium, liver and kidneys, with progressive impairment of renal
function and concentrating ability.
Spleen changes: in young children the spleen is enlarged, with occasional acute splenic sequestration.
Altered splenic function increases the risk of serious infection particularly meningitis sepsis caused by
pneumococci and haemophilus influenzae (polysaccharide encapsulated organisms). As the child ages, auto
splenectomy reduces spleen size. Cardiomegaly is invariably present in older children (sickle-related
cardiomyopathy), secondary haemosiderosis from increased iron absorption may damage liver, pancreas and
heart, and there may be gall stone formation. Puberty is frequently delayed, and chronic leg ulcers occur in
late adolescence.
79. Sickle cell trait is usually characterized by all of the following except:-
A. Red cell concentration of HbS is about 50%
B. Splenic infarction rarely occur except under extreme hypoxic condition
C. Reticulocyte count is usually elevated
D. In some individual hyposthnuria & hematuria may result from sickling in the renal medullary
capillaries
E. The incidence in American black is 1 in 12
Ans:- C
80. In paroxysmal nocturnal haemoglobinuria:-
A. The defect is a congenital abnormality of RBC production by the bone marrow.
B. Hamoglobinuria is a characteristic finding.
C. Venous thrombosis is a recognised complication.
D. The diagnostic test is Hams test.
E. Transforms into acute leukaemia in approx. 5%.
Ans:-BCDE
PNH is an acquired clonal defect of cell membranes that makes the cells more sensitive to lysis by
complement. This results in intravascular haemolysis and haemoglobinuria. Crises of haemolysis can be
triggered by infection, and chronic haemolysis may result in nephropathy. There is an increased risk of
venous thromboses (esp. in unusual sites such as saggital sinus or hepatic veins).The diagnostic test is
Hams test (acidified serum lysis). The disease may transform into acute leukaemia (in approx 5%).
81. The following conditions commonly require splenectomy in later life:
A. Sickle cell trait
B. Sickle cell disease
C. Beta Thalassaemia
D. Glucose-6-phosphate dehydrogenase deficiency
E. Idiopathic thrombocytopenic purpura true
Ans:- C
Comments:
Because of the risk of post-operative sepsis, splenectomy should be limited to specific indications. These
include:
Splenic rupture, anatomic defects.
Haemolytic anaemias, immune cytopenias.
Metabolic storage diseases.
Secondary hypersplenism.
Surgical indications (rare).
The major risk is infection, particularly in children less than 5 years. The risk of sepsis is slightly less in
splenectomies done for trauma, red cell membrane defects, and immune cytopenias then when there is a pre-
existing immune deficiency such as Wiskott-Aldrich Syndrome or reticuloendothelial blockage such as
storage diseases or severe haemolytic anaemias.
82. The following is true of aplastic anaemia:-
A. Congenital aplastic anaemia is more common than the acquired form
B. Fanconi's anaemia is the commonest cause
C. Age related anaemia is prerequisite for the diagnosis to be confirmed
D. Erythropoietin is an effective treatment in the majority of cases
E. Allogeneic bone marrow transplantation is recognized treatment option
Ans:- E
83. Aplastic anaemia:
A. Is defined as pancytopenia in the peripheral blood and blast cells in the bone marrow.
B. Has been associated with chloamphenicol eye drops.
C. Is preduced by hepatitis in 5-10% of cases
D. Is a feature of paroxysmal nocturnal haemoglobinuria
E. Can effectively be treated by growth factor injections
Ans:-CD
Aplastic anaemia is defined as the presence of pancytopenia in the peripheral blood and a hypocellular
marrow in which normal marrow is replaced by fat cells, abnormal cells are not found in either the
peripheral blood or bone marrow.
Causes include: drugs, viral infections and paroxysmal nocturnal hemoglobinemia.
The latter condition is an acquired defect of the red cell membrane which is associated with intravascular
hemolysis as the red cells are highly susceptible to complement lysis.
Many drugs have been implicated in aplastic anaemia but the evidence for many is slim.
Oral chloramphenicol not eye drops have been associated with aplastic anaemia, hepatitis, presumably of
viral origin is a precursor of aplastic anaemia in 5-10% of cases.
In the majority of cases no specific hepatitis virus is identified.
Definitive treatment is with immunosuppression or with allogenic stem cell transplantation in young patients
with HLA-matched doners.
Growth factors only have a role acutely to treat sever infection and are not effective as long term
treatment
84. These inborn errors have successfully been treated by bone marrow transplantation
A. Cystic fibrosis
B. Hurler's syndrome.
C. Gaucher's syndrome.
D. Fanconi's anaemia.
E. Chediak-Higashi syndrome.
Ans:-BCDE
85. Regarding leukocytes:-
A. Eosinophils play role in the defense against parasites
B. Mast cell are derived from basophils
C. Once migrated into the tissue, neutrophil is called a phagocyte
D. Lymphocytes contain myeloperoxidase
E. Band cells are immature neutrophils
Ans:-ABE
86. 5 year old child is found to have neutrophil count of 5110
9
/L this may be due to :
A. Acute liver failure.
B. Corticosteroid therapy.
C. Brucellosis.
D. Aplastic anaemia.
E. Hypersplenism.
Ans:-ABC
Granulocytes survive for only 612 hr in the circulation and, therefore, daily production of 2 10
4
granulocytes/L of blood is required to maintain a level of circulating granulocytes of 5 10
3
/L. In
contrast, lymphocytes can exhibit lifetimes measured in months or years and require daily renewal of
lymphocyte progenitors at rates substantially lower than the other hematopoietic progenitors. The mean
WBC count at birth is high, followed by a rapid fall beginning at 12 hr until the end of the 1st week.
Thereafter, values are stable until 1 yr of age. A slow, steady decline in the WBC count continues
throughout childhood until reaching the adult value during adolescence. Leukopenia in adolescents and
adults is defined as a total WBC count <4,000/L.
87. Neutropenia :-
A. Is defined as circulatory count < 510
9
/L.
B. Increase susceptibility to viral infection.
C. Can be caused by corticosteroid.
D. May be cyclical.
E. May be complication of folate deficiency.
Ans:-DE
Viral Bacterial Fungal Protozoal
Respiratory syncytial virus
Dengue fever
Colorado tick fever
Mumps
Viral hepatitis
Infectious mononucleosis
(EBV)
Influenza
Measles
Rubella
Roseola
Varicella
Cytomegalovirus
Human immunodeficiency
virus
Sandfly fever
Pertussis
Typhoid fever
Paratyphoid fever
Tuberculosis
(disseminated)
Brucellosis
Tularemia
Gram-negative sepsis
Psittacosis
Histoplasmosis
(disseminated)
Malaria
Leishmaniasis (kala-azar)
88. Atypical mononuclear cells are seen with :-
A. Toxoplasmosis.
B. Herpes simplex.
C. Rubella
D. Cytomegalovirus.
E. Epstein-Barr virus.
Ans:-CDE
89. Neutrophil
A. Are present in normal tissues.
B. Are directed to sites of inflammation by IL18.
C. Phagocytes cells more efficiency when opsonised with antibody and complement as
compared with antibody alone.
D. Kill phagocytosed organisms by releasing granule contents
E. Deficiency increases susceptibility to viral infections.
Ans :-BCD
Neutrophils remain in the blood stream and only enter tissues if there is inflammation (unlike macrophages)
to which they are principally directed by IL18. Antibody and complement is a much more efficient opsonin
than antibody alone. Neutrophils release toxic oxygen compounds (respiratory burst),nitric oxide,
phospholipases and proteases from granules to kill phagocytosed organisms. Deficiency or dysfunction of
neutrophils results in recurrent bacterial and fungal infections
90. The following can cause an eosinophilia:-
A. Penicillin
B. Hydralazine
C. Polyarteritis nodosa
D. Pneumocystic carini
E. Atopic dermatitis.
Ans:- ABCDE
Blood eosinophil numbers do not always reflect the extent of eosinophil involvement in disease-affected
tissues. The absolute eosinophil count, calculated as the white blood cell (WBC) count/L percent of
eosinophils, is usually <450 cells/L in the blood and varies diurnally, being more abundant in the early
morning and diminishing as endogenous glucocorticoid levels rise.
Eosinopenia occurs after corticosteroid administration and with some bacterial and viral infections , The
absolute eosinophilia count is used to quantitate eosinophilia. Many diseases are associated with moderately
severe (1,5005,000 cells/l) or severe (>5,000 cells/l) eosinophilia, Patients with sustained blood
eosinophilia may develop organ damage, especially cardiac damage as found in the idiopathic
hypereosinophilic syndrome, and should be monitored for evidence of cardiac disease. Many cases of
moderately severe eosinophilia often have no clear etiology.
ALLERGIC
DISORDERS
INFECTIOUS
DISEASES
MALIGNANT
DISORDERS
GASTROINTESTIN
AL DISORDERS
RHEUMATOLOGI
C DISEASE
IMMUNODEFI
CIENCY DISEASE
Allergic
rhinitis
Tissue-Invasive
Helminth Infections
Trichinosis
Toxocariasis
Strongyloidosis
Ascariasis
Filariasis
Schistosomiasis
Echinococcosis
Brain tumors
Inflammatory bowel
disease
Rheumatoid arthritis Hyper
Asthma Pneumocystis carinii
Hodgkin disease
and T-cell
lymphoma
Peritoneal dialysis Eosinophilic fascitis Wiskott
Acute and
chronic
urticaria
Toxoplasmosis
Acute myelogenous
leukemia
Eosinophilic
gastroenteritis
Graft vs host reaction
Pemphigoid Amebiasis
Myeloproliferative
disorders
Milk precipitin disease Omenn syndrome
Malaria Brain tumors
Chronic active
hepatitis
Pulmonary disease
Bronchopulmonary
aspergillosis
Hodgkin disease
and T-cell
lymphoma
Lffler syndrome
Coccidioidomycosis
Acute myelogenous
leukemia
Eosinophilic leukemia
Scabies
Myeloproliferative
disorders
Hypersensitivity
pneumonia
91. These statements are true:-
A. Methemoglobinemia is caused by oxidation of copper 2
+
to copper 3
+
form.
B. Methemoglobinemia may be due to HbC, abnormal hemoglobin.
C. Methemoglobin reductase maintain Hb in it's reduced state.
D. NADH is the energy source used in the redox reaction.
E. Production of NADH arise the consequence of conversion of 1, 3 diphosphoglycerate to
glyceraldehydes.
Ans : -CD
92. The following conditions present with purpura & normal platelet counts:-
A. Osler-Weber-Rendu syndrome
B. Ehler Danlos syndrome
C. BernardSoulier syndrome.
D. Wiskott-Aldrich syndrome.
E. Scurvy.
Ans:-ABE
93. The following are causes of thrombocytopenia:
A. B12 deficiency
B. Chronic myeloid leukaemia.
C. Splenectomy.
D. Bernard soulier syndrome
E. May-Hegglin anomaly.
Ans :-ABDE
B12 deficiency may be associated with a pancytopenia or with an isolated anaemia, thrombocytopenia or
leucopenia. Chronic myeloid leukaemia may be associated with a low, normal or high platelet count.
Bernard soulier syndrome and May Hegglin anomaly are congenital problems of platelet production and are
both associated with a thrombocytopenia. Splenectomy is associated with a thrombocytosis as well as
increased target cells and Howell Jolly bodies in the peripheral blood.
94. Thrombocytopenia occurs in
A pulmonary haemosiderosis.
B cavernous haemangioma.
C scurvy.
D infectious mononucleosis.
E chronic alcoholism.
Ans:- BDE
Comments:
A - blood picture of iron deficiency +/-eosinophilia
C - perifollicular haemorrhage due to vascular weakness and impaired platelet function
cavernous haemangioma - platelet sequestration
chronic alcoholism - suppression of megakaryocytes
infectious mononucleosis - immune mediated
Thrombocytopenia
II. Signs
A. Bleeding disorders
B. Purpura or Petechiae
III. Causes: Congenital
A. Glanzmann's Thrombasthenia (autosomal recessive)
1. Platelet membrane deficiency Glycoprotein IIb, IIIa
2. Defective binding of platelet Fibrinogen
3. Decreased platelet aggregation
B. Bernard-Soulier Disease (autosomal recessive)
1. Platelet membrane deficiency Glycoprotein Ib
2. Coagulation Factor X and Factor V deficiency
3. Large platelets and decreased platelet aggregation
C. Storage Pool Disease
1. Dense granule and/or alpha granule deficiency
2. Defective platelet release of ADP and Serotonin
IV. Causes: Acquired
A. Uremia
B. Chronic Liver Disease
C. Medications
1. Aspirin
2. Furosemide (Lasix)
3. Nitrofurantoin (Furadantin)
4. Heparin
5. Sympathetic blockers
6. Clofibrate (Atromid-S)
7. NSAIDs
95. The following are recognised causes of drug-induced immune thrombocytopenia:-
A. Chloramphenicol
B. Quinine
C. Penicillamine
D. Rifampicin
E. Heparin
Ans:-BDE
All the above drugs can cause thrombocytopenia. However chloramphenicol, penicillamine and
phenylbutazone cause this by bone marrow suppression. The others produce an antibody-drug-plasma
protein complex which deposits on the platelet surface leading to either lysis by complement or removal by
reticuloendothelial cells. Patients present with acute purpura and platelets often less than 10x10 9/l. Heparin
also causes thrombocytopenia through platelet aggregation.
96. Hemophilia A:-
A. Is the commonest inherited bleeding disorder.
B. Inheritance is autosomal dominant with variable penetrance.
C. Severity is determined by level of factor VIIIc
D. Is treated with factor VIII given by IM injection.
E. Is no longer treated with prophylactic factor VIII because of HIV risk.
Ans:-AC
Recombinant factor VIII is not widely available.
97. The following statement concerning hereditary bleeding disorder are correct :-
A. HCV is common cause of liver disease in adults with sever hemophilia treated before the
1990.
B. Antenatal diagnosis of hemophilia A is possible.
C. Von-Willebrand disease is inherited as an autosomal recessive manner in the majority of
patient.
D. Children with hemophilia have normal prothrombin time.
E. In hemophilia A , spontaneous bleeding into the joint occur when the factor VIII
concentration is reduced to 20% of normal.
Ans:-ABD
98. The following condition are caused by an abnormality of Hb synthesis :-
A. Beta thalassemia
B. Congenital spherocytosis
C. Pyruvate kinase deficiency
D. Hemophilia A
E. Sickle cell disease.
Ans:-AE
99. Von Willebrand disease (vWD):-
A. Is caused by pualitative or quantitative deficiency of von Willebrand factor
B. The resulting bleeding disorder in vWD is characterized by petechiae
C. The platelet count is normal
D. The ristocetin co-factor activity will be decreased
E. Is inherited in an X-linked recessive pattern
Ans:-ACD
100. Hemophilia:-
A. Doesn't cause coagulation disturbance in the newborn.
B. Lack positive family history in 1/3 of the affected boys.
C. Cause profound bleeding time.
D. Cause prolonged APTT ( test of intrinsic coagulation pathway).
E. Result from the synthesis of biologically inactive factor VIII (AHG).
Ans:-BDE
101. Which of the following is true of Factor VIII antihaemophilic globulin?
A. released mainly by megakaryocytes
B. mediates the endothelial platelet aggregation
C. is an essential co-factor in the activities of Factor X to Factor Xa
D. has a half life of 36 hours
E. deficiency is a major source of bleeding in Von Willebrand's disease
Ans:-BCDE
Comments:
factor VIII antihaemophilic globulin / factor = Von Willibrand's factor It is the plasma carrier for factor VIII
a-mainly from endothelium but also from megakaryocytes
b-it links platelet membrane receptors to vascular subendothelium
c-in association with calcium / activated factor IX
d-stored blood at 4C -> activity falls to 10% in first 3 days (Dr Shu Ho)
102. Regarding coagulation:-
A. All clotting factor are synthesized in the liver
B. Factor VIII is synthesized by hepatocytes and by endothelial cells
C. Factor CIII deficiency doesn't prolong the INR nor APTT
D. An isolated factor VII deficiency elevates the APTT only
E. Isolated factor II deficiency result in prolonged APTT, thrombin time( TT) and INR
Ans:-ABCE
Because factor XIII is responsible for the cross linking of fibrin to stabilize the fibrin clot, symptoms of
delayed hemorrhage are secondary to instability of the clot. Typically, patients have trauma 1 day and then
have a bruise or hematoma the next day. Clinical symptoms include mild bruising, delayed separation of the
umbilical stump beyond 4 wk, poor wound healing, and recurrent spontaneous abortions in women
103. The following are component of intrinsic coagulation:-
A. Prothrombine
B. Factore CP
C. Factore IC.
D. Fibrinogen
E. Factore VII
Ans:-BC
104. In neonatal alloimmune thrombocytopenia:-
A. The most commonly occurring human platelet antigene is HPA-1a
B. The father need to be HPA negative and the mother positive for the same HPA for NAIT to
develop
C. The first born child of genetically predisposed parents is commonly involved.
D. The thrombocytopenia become evident after approximetly 5 days of life.
E. The treatment includes antenatal periumbilical transfusion of maternal donated platelet.
Ans:-ACE
105. Neonatal thrombocytopenia is recognized feature of :-
A. SLE
B. Maternal ingestion of thiazid diuretic.
C. Maternal IgM antibodies.
D. In utero infection with Toxoplasmosis.
E. Shortened forearm.
Ans ;-ADE
106. Thrombocytopenia has recognized association with :-
A. HSP
B. Septicemia.
C. Rubella.
D. Von-Willebrand disease.
E. Portal hypertension.
Ans:- BCE
107. Idiopathic Thrombocytopenic Purpura (ITP) in Childhood:
A. Is usually self limiting.
B. Always requires treatment if the platelet count is less than 20 x 109/L.
C. Is associated with cerebral haemorrhage in less that 1% of cases.
D. Can be successfully treated with steroids.
E. Can be successfully treated with intravenous immunoglobulins.
Ans:- ACDE
Comments:
Acute idiopathic thrombocytopenic purpura is the most common of the thrombocytopenic purpuras of
childhood. It is associated with petechiae, mucocutaneous bleeding, and occasionally haemorrhage into the
tissues. There is a profound deficiency of circulating platelets despite adequate numbers of
megakaryocytes in the marrow. In 70% of cases there is an antecedent disease or viral infection. ITP has an
excellent prognosis even when no specific therapy is given. Within 3 months, 75% of patients recover
completely, mostly within 2 months. Spontaneous haemorrhage and intracranial bleeding (<1%) are usually
confined to the initial phase of disease. Thereafter, spontaneous manifestations subside. 90% of children
have a normal platelet count within a year, and relapses are unusual. FFP or platelet concentrates
have transient benefit only, but should be administered when life threatening haemorrhage occurs. When the
disease is mild and haemorrhages of the retina or mucus membranes are not present, no specific therapy is
given. Gammaglobulin and corticosteroid may be used in severe cases. The former produces a more rapid
effect, and the latter may disguise the occasional case of leukaemia presenting with thrombocytopenia.
108. In ITP:-
A. The purpuric rash is characteristically on the buttock and the extensor surface of the limbs.
B. Bone marrow examination characteristically reveals diminished numbers of megakaryocytes.
C. The infant of an affected mother may develop transient thrombocytopenia.
D. Steroid therapy is ineffective
E. The prothrombin time is prolonged
Ans :-C
109. Concerning ITP in childhood
A. Bed rest is indicated
B. Bone marrow examination is essential
C. Incidence is 1 in 1000 children per year
D. Platelet transfusion is necessary if the platelet count fall below 20
E. I/V immunoglobulin may be beneficial
Ans:-E
Antiplatelet antibodies bind to transfused platelets as well as they do to autologous platelets. Thus, platelet
transfusion in ITP is usually contraindicated unless life-threatening bleeding is present. (safe level of >20
10
9
/L,)
110. Idiopathic thrombocytopenia purpura:
A. Result from decreased platelet production.
B. Classically present with acute blood loss.
C. Require urgent treatment to prevent intracranial hemorrhage.
D. Should be confirmed by BM aspirate if there is no response to steroid therapy.
E. Is preceded by viral URTI in more than 60% of cases.
Ans:-E
111. ITP:-
A. Carries 1% risk of intracranial hemorrhage if the platelet count fall below 2010
9
/L
B. Should be treated by Splenectomy if the platelet count is<1010
9
/L.
C. Is due to impaired platelet production by the bone marrow.
D. Bone marrow examination is essential if immunoglobulin treatment is to be used.
E. Resolve spontaneously within 6 months in the majority of childhood cases
Ans:-AE
Splenectomy in ITP should be reserved for:-.
The older child (> 4 yr) with severe ITP that has lasted >1 yr (chronic ITP) .
Whose symptoms are not easily controlled with therapy is a candidate for splenectomy.
Splenectomy must also be considered when life-threatening hemorrhage (intracranial
hemorrhage) complicates acute ITP, if the platelet count cannot be corrected rapidly with
transfusion of platelets and administration of IVIG and corticosteroids.
Splenectomy is associated with a lifelong risk of overwhelming postsplenectomy infection caused by
encapsulated organisms.
112. Disseminated intravascular coagulation:
A. Is usually associated with a high fibrinogen level.
B. Is usually associated with a normal thrombin time.
C. May be associated with cytomegalovirus or varicella zoster infections.
D. May occur in liver disease.
E. May be associated with formation of microthrombi, so should be treated with intravenous
heparin
Ans:-CD
* Disseminated intravascular coagulation (DIC) may be caused by bacterial infections, in particular gram-
negative and meningococcal septicemia, by severe falciparum malaria infections and by viral infections
including CMV, HIV and varicella zoster.
* Liver disease can also cause DIC due to release of thromboplastins from damaged liver cells and reduced
concentrations of antithrombin and protein C.
* Investigations show a prolonged thrombin time, prothrombin time and activated partial thromboplastin
time (aptt), thrombocytopenia, a low fibrinogen and raised D-dimers or FDPs.
* DIC may be associated with microthrombin causing skin lesions, renal failure, gangrene or cerebral
ischemia but heparin therapy is usually not indicated as it can aggravate the bleeding tendency.
113. Feature of Wiskott-Aldrich syndrome include:
A. Autosomal recessive inheritance
B. Normal platelet count
C. T cell defect
D. Death from acute hemorrhage in 20%
E. Eczema
Ans:-CDE
Prenatal diagnosis and carrier detection is possible in 98%, Low serum IgM , normal IgG and raised IgA
&IgE, Examination of the bone marrow in WAS shows the normal number of megakaryocytes, although
they may have bizarre morphologic features. Transfused platelets have a normal life span. Splenectomy
often corrects the thrombocytopenia, suggesting that the platelets formed in WAS have accelerated
destruction. Approximately 5% of patients with WAS develop lymphoreticular malignancies. Successful
bone marrow transplantation cures WAS.
114. Neonatal thrombocytopenia is recognized feature of:-
A. SLE
B. Maternal ingestion of thiazide diuretics
C. Maternal IgM antibodies
D. In utero infection with toxoplasmosis
E. Shortened forearm.
Ans :-ADE
Neonatal thrombocytopenia often occurs in association with congenital viral infection, especially rubella and
cytomegalovirus; protozoal infection, such as toxoplasmosis; syphilis; and bacterial infection, especially
those caused by gram-negative bacilli, thrombocytopenia and abnormal abdominal findings is common in
necrotizing enterocolitis.
115. Neonatal thrombocytopenia:-
A. Is an indication for cerebral ultrasound
B. Is an absolute indication for platelet transfusion after delivery.
C. May be caused by toxoplasmosis infection during pregnancy
D. Can occur in infant born to mother with SLE
E. May be associated with absent radii
Ans:-ACDE
Neonatal isoimmune thrombocytopenia occurs in neonates who are platelet antigen (PLA1) positive born to
PLA1 negative mothers, sensitization occur in utero & first pregnancy can be affected. 2% of population is
PLA1 negative, 89% of population is PLA1 positive, but only 6% of PLA1 negative mothers with positive
fetuses will develop antibodies. 10% of affected fetuses will have intraventricular hemorrhage with high
associated morbidity and mortality. Monitoring of maternal antibodies can be performed during pregnancy
and platelet transfusion of PLA1 negative platelets given to infants at risk.
116. Glanzmann's thrombasthenia:-
A. Is inherited as an autosomal dominant
B. Gene locus is known
C. Platelet count is normal
D. Require long term steroid treatment
E. Splenectomy may be beneficial
Ans:-BC
117. In which of the following organ is Erythropoietin primarily produced:-
A. Bone marrow
B. Liver
C. Kidney
D. Spleen
E. Intestine
Ans:- C
118. Regarding the red cell:
A. Carbon Dioxide binds with reduced haemoglobin.
B. The oxygen affinity of haemoglobin is decreased in the presence of acidosis.
C. The oxygen affinity of fetal haemoglobin is greater than adult haemoglobin.
D. Carbonic anhydrase is present in all red cells.
E. Most carbon dioxide in venous blood is transported bound to albumin.
Ans:-ABCD
Comments:
Carbon dioxide is carried in the blood in 3 forms:
Dissolved (10%).
Bicarbonate, whose formation is encouraged by carbonic anhydrase present in
the red cell.
As carboamino compounds: hydrogen ions liberated from the bicarbonate
reaction are bound to haemoglobin which encourages the release of oxygen, since reduced
haemoglobin is less acid than the oxygenated forms.
Thus, the presence of reduced haemoglobin in the peripheral blood helps with the loading of carbon dioxide,
while the oxygenation which occurs in the pulmonary capillary assists in the unloading of it. The fact that
the deoxygenation of the blood increases its ability to carry carbon dioxide is often known as the Haldane
effect. Fetal haemoglobin contains g polypeptide chains in place of the b-chains of Hb A. Its resistance to
denaturation by strong alkali is used in its quantitations. Hb F is the predominant haemoglobin from 8 weeks
gestation, and constitutes 90% of the total haemoglobin of the 6 month fetus. At birth 70% of the total is Hb
F, and synthesis decreases rapidly postnatally, such that by a year, only 2% is present. Hb F has a greater
oxygen affinity than Hb A, so the growing fetus is preferentially ourished by oxygen in utero.
119. Hemoglobin
A. At 40 week gestation is 70-75% HbF
B. At 40 week gestation is 20-25% HbA
C. After age 3 is over 98% HbA
D. After age 3 year is approximately 3% HbF
E. In beta thalassemia minor contain elevated HbF & HbA
2
Ans :-ACE
Two hemoglobin gene clusters are involved in the production of hemoglobin and are located at the end of
the short arm of chromosomes 16 and 11, respectively. On chromosome 16, there are 3 genes within the
alpha () gene cluster: zeta () and 2 genes (
1
,
2
). On chromosome 11, there are 5 genes within the beta
() gene cluster: epsilon (), delta (), beta (), and 2 gamma () genes. The order of gene expression within
each cluster roughly follows the order of expression during the embryonic period, the fetal period, and
eventually childhood.
8 wk of fetal life, the embryonic hemoglobin are formed: Gower-1 (
2
2
), Gower-2 (
2
2
), and Portland
(
2
2
). In embryos of 48 wk gestation, the Gower hemoglobins predominate, but by the 3rd month they
have disappeared.
At 9 wk of fetal life, the major hemoglobin (Hb) is Hb F (
2
2
). Its resistance to denaturation by strong
alkali is the basis for determining the presence of fetal RBCs in the maternal circulation (the Kleihauer-
Betke test). After the 8th gestational wk, Hb F is the predominant hemoglobin; at 24 wk gestation it
constitutes 90% of the total hemoglobin. During the 3rd trimester, a gradual decline occurs, so that at birth
Hb F averages 70% of the total. Synthesis of Hb F decreases rapidly postnatally, and by 612 mo of age
only a trace is present.
At approximately 1 mo of fetal life, Hb A (
2
2
) appears, but does not become the dominant hemoglobin
until after birth, when Hb F levels start to decline. A minor hemoglobin, Hb A
2
(
2
2
) appears shortly before
birth and remains at a low level after birth. The final hemoglobin distribution pattern that occurs in
childhood is not achieved until at least 6 mo of age, and sometimes later.
The normal hemoglobin pattern is >95% Hb A, 3.5 Hb A
2
, and <2.5% Hb F.
Some Hb A (
2
2
) can be detected in even the smallest embryos. Accordingly, it is possible as early as 16
20 wk gestation to make a prenatal diagnosis of major -chain hemoglobinopathies, such as thalassemia
major . Prenatal diagnosis is based on techniques that examine the rates of synthesis of chains or the
structure of newly synthesized chains. Earlier diagnosis is possible using molecular biology techniques
and sampling of chorionic villus tissue or amniotic fluid if DNA structural defects are a cause of the
hemoglobinopathies. Gene deletion disorders such as the - thalassemias can be detected using the same
method.
By the 24th wk of gestation, 510% of Hb A is present. A steady increase follows, so that at term, Hb A
averages 30%. By 612 mo of age, the normal Hb A pattern appears. The minor Hb A component Hb
A
2
contains delta () chains and has the structure
2
2
. It is seen only when significant amounts of Hb A are
also present. At birth, <1.0% of Hb A
2
is seen, but by 12 mo of age the normal level of 2.03.4% is attained.
Throughout life, the normal ratio of Hb A to A
2
is about 30:1.
120. hemoglobin;
A. Is present in blood cells by the 14
th
day after gestation.
B. Is predominantly produced in the liver at term.
C. At term is mainly fetal hemoglobin.
D. Production increase after delivery.
E. Concentration at birth is 10-12 g/dl
Ans:- AC
Mesoblastic hematopoiesis occurs in extraembryonic structures, principally in the yolk sac, and begins
between the 10th and 14th days of gestation. By 68 wk of gestation the liver replaces the yolk sac as the
primary site of blood cell production, and by 1012 wk extraembryonic hematopoiesis has essentially
ceased. Hepatic hematopoiesis occurs in the liver throughout the remainder of gestation, although
production begins to diminish during the second trimester as bone marrow (myeloid) hematopoiesis
increases. The liver remains the predominant hematopoietic organ through wk 2024 of gestation
121. The O2-Hb dissociation curve is displaced to the right in:
A. Fetal Hb
B. Methemoglobinemia
C. Increased lactic acid production
D. Chronic hypoxia due to cyanotic conditions
E. Increased 2,3 DPG
Ans:-CDE
When shifted to the right, O2 is easily given up and this will be beneficial in conditions when the body is
working hard.
* Increase in CO2 production due to increased metabolism
* Increase in H+
* Hyperthermia
* Chronic hypoxic states/anemia, where there is an increase in 2,3 DPG
122. en uptake by haemoglobin is characterized by:
A. Alkalosis.
B. Raised temperature.
C. Increased 2-3 DPG.
D. Increased red cell volume and MCH.
E. High altitude.
Ans:-BCE
99% of the oxygen which dissolves in blood is transported by the oxygen carrying compound haemoglobin.
Haemoglbin is composed of four globin subunits (in normal adults two alpha and two beta globin chains)
each of which contains a haem group. Haem is a complex of prophyrin and ferrous iron. The oxygen-
haemoglobin dissociation curve relates the saturation of haem with oxygen as a % (the y axis) to the PO2 in
mmHg(on the x axis). This curve is affected by a number of factors:
1- PH a fall in pH shifts the curve to the right i.e a higher PO2 is required for haemoglobin to bind
oxygen. This is known as the Bohr effect. An increase in CO2 (as is found in peripheral tissues) will
cause a fall in pH, and the associated decrease in affinity of haemoglobin for oxygen contributes to
the transfer of oxygen from the blood in to the tissues. Alkalosis will have the opposite effect.
2- Temperature A rise in temperature shifts the curve to the right i.e oxygen uptake by haemoglobin is
decreased.
3- 2,3-DPG levels- this substance is formed by glycolosis via the Embden-Meyerhof pathway. It binds
to the beta-globin chains of deoxyhaemoglobin and causes oxygen to dissociate from Hb. A rise in
2,3-DPG shifts the oxygen dissociation curve to the right releasing more O2.
4- High altitude results in a rise in 2,3-DPG concentrations in RBCs, with an increase in the availability
of oxygen to the tissues. The greater affinity of foetal haemoglobin for oxygen as compared to its
adult counterpart is a result of the decreased binding of 2,3-DPG by the foetal gamma globin chain.
5- With regards RBC indices, it is the MCHC which affects the oxygen-dissociation curve, rather than
the MCH or MCV. In anaemia, the MCHC is reduced, and the curve is right shifted, increasing the
release of oxygen to the tissues.
123. Hazard of blood transfusion include :-
A. Haemosiderosis.
B. Circulatory failure.
C. Hypokalemia.
D. DIC
E. Cytomegalovirus infection.
Ans:-ABDE
124. Red cell physiology
A. Erythropoietin is produced predominantly by endothelial cells, in response to reduction of
partial pressure of oxygen in the blood stream
B. Vitamin C is coenzyme required in the initial stage of haem synthesis .
C. The mitochondria & ribosomes within the erythroblast produce haem & globin respectively
D. Molecule of hemoglobin is comprised of four globin chains attached to their own haem moiety
E. The red cell generates its own energy by metabolizing glucose only
Ans:-CDE
125. Arterial thromboses are associated with:-
A. Behcets syndrome
B. Atrial fibrillation
C. Factor V Leiden mutation
D. Protein C deficiency
E. Polycythaemia
Ans:-ABE
Arterial thromboses may be associated with Behcets syndrome, atrial fibrillation and polycythaemia. Factor
V Leiden mutation and protein C deficiency are associated with venous thromboses
126. Which of the following is not a recognised complication of anticoagulation therapy?
A. Deep venous thrombosis.
B. Thrombocytopaenia.
C. Skin necrosis.
D. Osteoporosis.
E. Peripheral neuropathy.
Ans:-E
Although the main side effect of anticoagulation therapy is excessive bleeding, more unusual side effect
exist.
Heparin may be associated with both thrombocytopaenia and thrombosis as part of the disorder known as
heparin-indused thrombocytopaenia (HITT). This is an acquired immune disorder in which heparin-
dependant anti-platelet antibodies form, leading to sever thrombocytopaenia and occasionally catastrophic
thrombosis. Treatment involves stopping heparin and anticoagulating with hirudin or danaparoids. Long-
term heparin therapy may be associated with osteoporosis.
Warfarin, especially induction therapy in patients with inherited protein C or S deficiency, may be
associated with skin necrosis.
127. Low protein C levels are not seen with:
A. Warfarin therapy
B. Neonatal purpura fulminans
C. Factor V Leiden
D. Chicken pox
E. Meningococcal septicaemia
Ans:- C
Protein C is a vitamin K-dependent protein synthesised in the liver. It circulates in an inactive form, which
becomes activated by thrombin/thrombomodulin complexes on the surface of endothelial cells. Activated
protein C inhibits coagulation by degrading coagulation factors Va and VIIIa and resulting in decreased
thrombin formation. It requires protein S as a cofactor.
Causes of protein C deficiency are:
1) Congenital
* homozygous deficiency - those with severe deficiency - (protein C levels <1% of normal) present with
severe neonatal purpura fulminans, cerebral thrombosis and DIC. Those with milder defects have protein C
levels of 10 -24 % of normal. They can present with massive venous thrombosis as older children.
* heterozygous deficiency (protein C levels 30-40% of normal) most frequently presents after puberty with
deep venous thrombosis of the lower limb. It can also present with recurrent superficial thrombophlebitis
and DVT. Most thrombotic episodes occur spontaneously but known associated risk factors include surgery
with immobilisation, pregnancy and the oral contraceptive.
Many people remain asymptomatic. A positive family history of thrombosis is associated with an increased
risk of symptoms - + - 50% of individuals with heterozygous protein C deficiency and a family history of
thrombosis, will experience a thrombotic event.
2) Causes of acquired protein C deficiency:
* infections - meningococcal disease and varicella infection
* DIC
* hepatic disease
* drugs - warfarin, chemotherapeutic agents e.g. cyclophosphamide, methotrexate, 5-fluorouracil), l-
asparaginase
* malignancy - acute myeloid leukaemia
* chronic inflammation - inflammatory bowel disease
* haemolytic uraemic syndrome
* thrombotic thrombocytopaenic purpura
Patients with protein C deficiency are at high risk for warfarin-induced skin necrosis during initiation of
therapy with warfarin.
Factor V Leiden refers to a point mutation in the gene for factor V results in resistance of factor V to the
actions of activated protein C, and consequently a thrombophillic state. Protein C levels are not decreased
QUESTION NUMBER 4637
Patients with iron overload may have endocrine dysfunction. Endocrine dysfunction in patients with iron
overload is likely to spare which of the following organ functions:
a) Pituitary function
b) islet cell function
c) thyroid function
d) ACTH production
e) parathyroid activity
Correct answer: C
EXPLANATION
Dysfunction of the endocrine pancreas is common in patients with iron overload. Some people develop overt
diabetes mellitus requiring insulin therapy. The disturbances in carbohydrate metabolism are often more
subtle, however. An oral glucose tolerance test often unmasks abnormal insulin production. Vigorous
exorcism of the excess iron occasionally reverses the islet cell dysfunction (Bomford and Williams, 1976).
Exocrine pancreatic function, in contrast, is usually well-preserved.
Pituitary dysfunction produces a plethora of endocrine disturbances (Costin et al., 1979). Reduced
gonadotropin levels are common. When coupled with primary reductions in gonadal synthesis of sex
steroids, this phenomenon delays sexual maturation in some children with transfusional iron overload.
Secondary infertility is common (Schafer et al., 1981). Although Addison's syndrome is uncommon with
iron overload, production of ACTH is occasionally deficient. A metapyrone stimulation test shows delayed
or diminished pituitary secretion of ACTH (Schafer et al., 1985).
Thyroid function is usually well-preserved in patients with iron overload. In contrast, parathyroid activity is
frequently compromised. Functional hypoparathyroidism can be demonstrated in many patients by inducting
hypocalcemia with an intravenous bolus of ethelyenediamine tetraacetic acid (EDTA) while monitoring the
production of parathyroid hormone
The most typical finding of severe folate deficiency in a blood smear :
a) fragmentation of the cytoplasm of megakaryocytes
b) lobulated macrocytosis
c) Hypersegmentation of neutrophils
d) megaloblastic erythroblasts
e) giant metamyelocytes
Correct answer: C
EXPLANATION
The blood film is likely to show oval macrocytes and hypersegmented neutrophil nuclei (with six lobes). In
severe cases, the white cell count and platelet count also fall (pancytopenia). The bone marrow shows
characteristic megaloblastic erythroblasts and giant metamyelocytes (early granulocyte precursors).
Biochemically, there is a increase in plasma of unconjugated bilirubin and serum lactic dehydrogenase, with,
in severe cases, an absenc of haptoglobins and presence in urine of haemosiderin.
The features of both B12 and folate deficiency include an Elevated MCV and low reticulocyte count.
However, many have normal indices because of coexistent thalassemia or iron deficiency, etc.
RBC folate level is preferred because serum folate level varies with meals and is an unreliable indicator of
base state. Thrombocytopenia (50%) and leukopenia are late findings.
Smear shows anisocytosis, poikilocytosis, basophilic stippling, and hypersegmentation of neutrophils. Once
the diagnosis of vitamin B12 deficiency is made, a Schilling test can identify the etiology, although it is
optional.
a water soluble ferric heme containing enzyme that reduces hydrogen peroxide to water.
a) malate dehydrogenase
b) Cytochrome c peroxidase
c) 5-alpha reductase
d) protein C
e) pepsin
Correct answer: B
EXPLANATION
water soluble ferric heme containing enzyme in the peroxidase family of enzymes that takes reducing
equivalents from cytochrome c and reduces hydrogen peroxide to water.
QUESTION NUMBER 942
A 13 year old female has swollen cervical lymph nodes and is febrile. Her blood culture is positive for
staphylococcus aureus. She shows other signs of being septic. The intern attending her tells her associate
that the patient's WBC count is 15,000 cc/mm with a shift to the left.
What does she mean by a 'shift to the left?'
a) increased number of white blood cells in the peripheral blood
b) decreased number of white blood cells in the peripheral blood
c) hypersegmented granulocytes present in the peripheral blood
d) increased number of immature white blood cells in the peripheral blood
e) increased number of immature red blood cells in the peripheral blood
Correct answer: D
EXPLANATION
A shift to the left refers to an increase number of immature white blood cells in the peripheral smear.
Typically, the immature white blood cells are being released prematurely by the bone marrow in order to
combat some sort of infection.A increased number of white blood cells in the peripheral blood (choice a) is
called a leukocytosis.A decreased number of white blood cells in the peripheral blood (choice b) is called a
leukopenia.
Hypersegmented granulocytes present in the peripheral blood (choice c) is a shift to the right.An increased
number of immature red blood cells in the peripheral blood (choice e) is a reticulocytosis.
A 12 year old boy with known sickle cell with a history of recurrent painful episodes in the arms, hands and
legs complains of hip pain. The pain is exerbated by weight bearing. On examination he is noted to have a
limp. The most likely diagnosis is
a) Acute hemarthrosis
b) Osteonecrosis
c) Baker's cyst
d) pigmented villonodular synovitis
e) rheumatoid arthritis
Correct answer: B
EXPLANATION
Osteonecrosis, also called avascular or ischemic necrosis, results from bone death secondary to an impaired
blood supply. The femoral head is commonly involved, leading to hip-joint arthritis, but any other joint can
be affected. Symptoms include pain, especially when bearing weight, limping and the eventual restricted
movement of the involved joint. Osteonecrosis should be considered in young people with risk factors such
as corticosteroid use, hemoglobinopathies or sickle cell disease, as well as in alcoholic and elderly diabetic
patients

Serum Iron Levels and Iron Deficiency Anemia in Children

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Serum Iron Levels and Iron Deficiency Anemia in Children

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ABDULRAHMAN BASHIRE CHILDREN HOSPITAL -- BENGHAZI 1

1. The serum iron is often raised in the following condition:-

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