HEMOLYTIC ANEMIA

Hemolytic anemia arising from the increased destruction of cells of erythroid series. They may
be inherited or acquired, with intravascular hemolysis or intracellular type, associated with
internal abnormalities of red blood cells themselves or by external influences.

Hemolytic anemia associated with internal

abnormalities of erythrocytes
MEMBRANOPATII

The most frequent variant membranopaty in Russia - the ancestral sphere-rotsitoz, or disease
Minkowski-Chauffard. Prevalence of hereditary spherocytosis - about 1:5000 without light and
asymptomatic forms of the disease.

Etiology and pathogenesis. In 70% of cases the disease Minkowski-Chauffard - 5R, 30% - is
the result of spontaneous mutations or river. The basis of the disease is a molecular defect in the
erythrocyte membrane proteins, lowering their osmotic resistance. The longevity of erythrocyte
reduced to 8-10 days, the defective cells are destroyed in the spleen.

The clinical picture is diverse - from asymptomatic to life-threatening hemolytic

anemia. Characteristic triad of symptoms: jaundice, splenomegaly, anemia. There are often
delayed physical development, abnormalities of the skull and facial skeleton. The blood analysis
reveal the decrease in concentration of hemoglobin, reticulocytosis, mikrosferotsitoz, the
displacement curve Price-Jones left. Reduced osmotic resistance of erythrocytes. The course of
moderate and severe forms of disease may be accompanied by hemolytic and aplastic crisis.

Hemolytic crisis occurs spontaneously or on background infection: jaundice appears or gets

worse, there is increasing the size of the spleen, it becomes painful. In the blood, decreasing the
concentration of hemoglobin content and red blood cells, reticulocytosis is growing, increasing
the concentration of indirect bilirubin and LDH. The first hemolytic crisis, atypical and rarely
diagnosed, can develop in the neonatal period. aplastic crisis usually provokes parvovirus
infection (Parve virus B19), leaving strong immunity, and therefore such a crisis occurs in
patients once in a lifetime. The rapidly decreasing the concentration of hemoglobin and the
erythrocyte contents, sometimes - white blood cells and platelets. Content of bilirubin and the
number of reticulocytes did not increase. The condition of patients is often difficult because of
developing hypoxemia and hypoxia. Relatively frequently in patients with hereditary
spherocytosis developing gallstone disease.

Treatment. Outside the crisis in the mild and moderate forms of treatment is not carried
out. Hemolytic crisis require infusion therapy, transfusion of packed red blood cells at lower
concentrations of Hb less than 70 g / liter. Splenitis-ectomy is indicated for severe or
complicated by moderate form. Before the operation, it is desirable to vaccinate children
polyvalent pneumococcal and meningococcal vaccines, and Haemophilus influenzae vaccine,
since after splenectomy patients are at risk of more frequent and severe viral and bacterial
infections. Children under 6 years of age, preferably an alternative operation -
rentgenendovaskulyarnoy occlusion of blood vessels of the spleen, the remaining functions body
and to reduce hemolysis.

ENZIMOPATII

The most common variant enzimopaty, leading to the development of hemolytic anemia -
deficiency of glucose-6-phosphate dehydrogenase-dehydrogenase. The disease is widespread
among African Americans (6-30%), less - among the Tatars (3.3%), Dagestan (5-11,3%) in the
Russian population reveal rare (0.4%). Special case of deficiency of glucose-6-phosphate
dehydrogenase - favizm. Hemolysis develops by eating fava beans, kidney beans, peas,
inhalation of naphthalene dust.

Etiology and pathogenesis. Inherited deficiency of glucose-6-phosphate dehydrogenase (X),

which makes most sick men. There were about 400 million carriers of the abnormal gene. The
disease develops, usually after taking certain drugs [nitrofuran derivatives, quinine, isoniazid,
ftivazid, aminosalicylic acid (sodium para-aminosalicylate), nalidixic acid, sulfonamides, etc.] or
on the background of infection.

Clinical picture. The disease is manifested rapid development of hemolysis in the use of the
above substances or infections (especially pneumonia, typhoid fever, hepatitis). The lack of
gluco-so-6-phosphate dehydrogenase deficiency can cause neonatal jaundice. The blood analysis
reveal reticulocytosis, increased direct and indirect bilirubin, LDH, alkaline phosphatase. The
morphology of erythrocytes and erythrocytic indexes are not changed. Diagnosis is based on the
results of determining the activity of the enzyme.

Treatment. Outside crisis treatment is not carried out. When fever used physical methods of
cooling. In chronic hemolysis prescribe folic acid 1 MT / day for 3 weeks every 3 months. When
crisis cancel all medicines, infusion therapy on the background of dehydration.

HEMOGLOBIN

Hemoglobin - a group of hereditary diseases caused by disorders of synthesis or structure of

hemoglobin. They are characterized by inefficient erythrocytes-ropoezom due to violation of
utilization of intracellular iron for the synthesis of hemoglobin.

Thalassemia

Thalassemia - the most common heterogeneous group of hemoglobin, found mostly in

Mediterranean countries, in the East and the North Caucasus.
Etiology and pathogenesis. The basis of the disease is a genetic defect (p). The excess of
unpaired globin chains induces the formation of insoluble tetramers, adsorbed to the membranes
of red blood cells and damaging them.

Clinical picture. In heterozygous carriers of the gene or mild disease does not occur at all. In
homozygous carriers of the gene and in combined forms, develops a severe form of thalassemia
(large Talas-semiya, Cooley anemia). In the latter case, anemia detected in the first months of
life. Patients usually have a lot of dysembryogenic stigmas (Mongoloid cut eye slits, hyperplasia
of the hillocks of the skull, low growth), skin hyperpigmentation, enlarged and tightened the
liver and spleen. This is due to the emergence of foci of extramedullary hematopoiesis and a
progressive n-mohromatozom. Often develop pericarditis, cirrhosis, hypersplenism and
decreased immunity. Without treatment, patients die at an early age.

Laboratory and instrumental investigations. The blood analysis reduced the concentration of
hemoglobin and erythrocyte content (mishenevidnyh with basophilic granules). Characteristic
erythroblastosis and mikrotsitoz. Reduced concentration of NA, increased - HbF and
NA 2. Increased osmotic resistance of erythrocytes and the concentration of indirect
bilirubin. The concentration of serum iron is normal or elevated. Total iron binding capacity of
serum in the normal range. The coefficient of saturation transfer width of iron significantly
improved. Detects desferalovuyu siderouriyu. The radiographs reveal characteristic changes of
the skull in the form of cross-striation ( "brush") and osteoporosis, and pathological fractures of
long bones.

Treatment of mild symptomatic. Assign folic acid in courses for 3 weeks every 3 months, 1-2
mg / day. At moderately forms transfusion washed red blood cells as needed, conduct courses
deferok-Samina for removing excess iron and splenectomy. In severe thalassemia, the only
radical method of treatment - the transplantation of red bone marrow. Examines the effectiveness
with thalassemia cytostatics, epoetin beta (for example, recombinant human erythropoietin).

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Sickle cell anemia

Sickle-cell disease - is often recorded by a hereditary haemoglobinopathy characterized by

moderate chronic hemolytic anemia, recurrent acute painful crises and increased susceptibility to
infectious diseases (mainly attributable to Streptococcus pneumoniae). The disease is diagnosed
in 1% of African-Americans, often recorded in Azerbaijan and Dagestan.

Etiology and pathogenesis. The disease is associated with the presence of pathological
gene HbS (^). HbS polymerizes with the formation of long chains, alter the shape of red blood
cells (which are crescent-shaped). Sickle-shaped red blood cells cause an increase in the
viscosity of blood stasis, to create a mechanical barrier in the small arterioles and capillaries,
leading to tissue ischemia (associated with pain than crises). In addition, sickle-shaped red blood
cells less resistant to mechanical stress, which leads to their hemolysis.
Clinical picture. Appear moderate jaundice, trophic ulcers of the ankles, retarded physical
development (especially boys), priapism, predisposition to aplastic and hemolytic crises, pain
crises, splenomegaly, cholelithiasis, avascular necrosis, osteonek-roses with the development of
osteomyelitis.

Laboratory studies. Electrophoresis Hb exhibit poly-merizovanny HbS. The blood analysis

reveal the decrease in concentration of hemoglobin, reticulocytosis (giperregeneratornaya
anemia), leukocytosis, thrombocytosis, reduced ESR. In blood smears of peripheral blood
erythrocytes have a sickle shape. Concentration of serum bilirubin slightly increased. In the red
bone marrow - erythroid hyperplasia, increased number of multi-cell precursors.

Treatment. In acute pain crises moderately outpatient use Non-narcotic analgesics (ibuprofen,
paracetamol), in acute pain crises in stationary conditions - parenteral narcotic analgesics. In the
development of infectious complications prescribe antibiotics. Supportive therapy - the
transfusion of washed or frozen red blood cells, as well as anticoagulants. Anemia radically
curable. Some children die of necrotic complications or sepsis.

Hemolytic anemia associated with external influences

Autoimmune hemolytic anemia

Autoimmune hemolytic anemia - the most common variant of this group of

anemias. Autoimmune hemolytic anemia develops as a result of immune conflict - education to
AT change in the surface Ag own red blood cells. Formed complexes "erythrocyte-antibody" or
subjected to agglutination with each other, with little exposure to cold (intravascular hemolysis),
or absorbed by macrophages in the spleen (intracellular hemolysis).

Classification

The classification is based on the difference of autoantibodies.

Heat AT (react with red blood cells at body temperature not less than 37 C).

A cold AT (react with red blood cells at temperatures below 37 C).

Two-phase hemolysins (fixed on the red blood cells during cooling of any part of the body and
cause hemolysis with subsequent increase in body temperature to 37 C).

The prevalence of autoimmune hemolytic anemia is 1:75 000-1:80 Ltd., with 80% of patients of
any age, it is caused by thermal AT (agglutinin). The children in 79% of cases they are directed
against the Rh-Ar. Rarely observed anemia with cold AT, often after a viral infection in infants
and the elderly. Autoimmune hemolytic anemia can be either idiopathic or symptomatic (with
viral infections, lymphoproliferative diseases, histiocytosis, medication).
Clinical picture

The disease often begins with acute hemolytic crisis with a combination of intracellular and
intravascular hemolysis. The patient's body temperature rises suddenly appear weakness,
shortness of breath, tachycardia, yellow-tushnost skin and mucous membranes, brown or brown
staining of urine, increased liver and spleen. With a gradual onset of the disease occur arthralgia,
abdominal pain. The blood analysis reveal normo-or hyper-perhromnuyu anemia, anisocytosis,
small reticulocytosis. Perhaps the development normoblastoza, spherocytosis, thrombocytopenia,
leukocytosis with a shift orthocytosis left.Increasing ESR. Coombs positive sample (sample
reveals complex erythrocyte-AT ").

Treatment

Basic principles of treatment: blocking the synthesis of autoantibodies (Predn-zolon, cytostatics,

cyclosporine), restricting access to the AT target cells (of high-purified Ig, prednisolone),
stimulation of regeneration of blood cells (folic acid). Applied and operative treatment (splenitis-
ectomy). If you want to spend the transfusion of washed red blood cells.

MECHANICAL microangiopathic hemolytic anemia

The origin of these groups of hemolytic anemia associated with traumatic fragmentation of
erythrocytes. The causes of mechanical failure may be the following factors.

Stenosis or aortic aneurysm.

Narrowing or obstruction of the small blood vessels (DIC, hemolytic uremic syndrome,
Moshkovich, systemic vasculitis, kidney and liver, streptococcal pneumonia).

Damaged heart valves and major vessels (prosthetic valves).

Running long distances, classes contact sports, etc. The main reasons for the fragmentation of
erythrocytes in small vessels: damage to the vessels themselves, the formation of blood clots and
fibrin strands in the bloodstream.

Clinical picture

The clinical picture dominated by jaundice and haemorrhagic syndrome. In the analysis of blood
concentration of reduced hemoglobin content of red blood cells and platelets. Pronounced
anisocytosis, poikilocytosis, mikrosferotsitoz reveal "subulate", "helmet" cells and fragments of
red blood cells. In the biochemical analysis of blood - increased LDH, indirect bilirubin, serum
iron may decrease due to loss of its excretion. In urine - gemoside-rinuriya, rarely -
hemoglobinuria. Direct Coombs test negative. Differential diagnostic criteria - a combination of
hemolytic anemia, fragmented red cells, leukocytosis and thrombocytopenia.
Treatment

Treatment of mechanical hemolytic anemia involves treating the underlying disease, as well as
plasmapheresis, transfusion of large doses of fresh frozen plasma, if necessary - erythrocyte
mass. Developing new methods of treatment using high doses of intravenously injected Ig (400
mg / kg / day and more) and inhibitors of apoptosis.

ANEMIA IN OTHER DISEASES

Anemia in other diseases (chronic infectious, systemic, cancer, etc.) - secondary syndromes, are
regarded as adaptive and characterized by decreased production of red blood cells and a violation
of iron reutilization.

The clinical picture associated with major disease, anemia does not determine the state of health
and condition of the patient. It usually appears late, slowly developing and progressing in the
future. In the analysis of blood moderately reduced the concentration of hemoglobin, the 1 / 4
patients exhibit mikrotsi-inos and gipohromiyu, especially if severe underlying disease. Other
changes in red blood cells are absent. Possible leukocytosis and toxic granularity of neutrophils.
Reduced concentration of serum iron and total iron binding capacity of serum, moderately
increased concentration of serum ferritin.Concentration of erythropoietin does not match the
degree of anemia. Identify biochemical signs of active inflammation (increased C-reactive
protein, fibrinogen, ESR). Anemia refractory to iron therapy.

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