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Platelet count Normal range
PBS Normocytic, normochromic, Few
spherocytes, increase in
neutrophilic bands, few early WBCs,
3 nucleated RBCs
Urinalysis 3+ blood
Urine and sputum cultures negative
chemistry profile elevated levels of total bilirubin,
alkaline phosphatase, lactate
dehydrogenase, and aspartate
aminotransferase
direct antiglobulin test (DAT) positive
indirect antiglobulin test negative
eluate Negative
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m 
A 55 year old man sought a medical
attention for the onset of chest
pain. Physical examination revealed
Y   
 Y    . The
past medical history included
 Y  Y, and there was a family
history of anemia.
A complete blood count
(CBC) yielded the following
results:
v        

 
ãBC (x 109/L) 13.4 4.5-11

RBC (x 1012/L) 4.28 4.3-5.9

Hemoglobin (g/dL) 11.7 13.9-16.3

Hematocrit (%) 32.5 39-55

MCV (fL) 76 80-100

MCH (pg) 27.3 25.4-34.6

MCHC (g/dL) 36 31-37

RDã (%) 22.9 11.5-13.5


    Y 
   
 slight anisocytosis
 slight polychromasia
 several dark, round
microspherocytes lacking
central pallor
The platelet count and
platelet distribution on the
smear were normal.
A 
A

!rom the data given, what is


your initial diagnostic
assessment of the anemia?
A

!rom the data given, what is


your initial diagnostic
assessment of the anemia?

   Y  
 YY
A
ãhat additional laboratory tests
would be of value in establishing
the diagnosis, and what
abnormalities in these tests would
be expected in confirming your
impression?
A
ãhat additional laboratory tests would be of value in establishing the
diagnosis, and what abnormalities in these tests would be expected in
confirming your impression?

† ·       ´ An increased


bilirubin level would be expected

†  


 ´ An increased
reticulocyte count

† · Y        ´ A decreased


or absence of haptoglobin
A
ãhat additional laboratory tests would be of value in establishing the
diagnosis, and what abnormalities in these tests would be expected in
confirming your impression?

† Y 
   Y ´ An increased
Osmotic fragility test

† m   m  Y ´ Abnormal (15


days)

† !
      ´ Presence of
urobilinogen because of destruction of
ertythrocytes
A
ãhat additional laboratory tests would be of value in establishing the
diagnosis, and what abnormalities in these tests would be expected in
confirming your impression?

† v
    Y v
Test ´ An
increased LDH level

† m Y  Y ´ A negative Coombs test

†  
    YY  Y ´ An
increased autohemolysis test and
corrected by the addition of glucose.
A
ãhat is the cause of this type
of anemia?
A
ãhat is the cause of this type of anemia?

Hereditary spherocytosis is due to the


presence of an autosomal dominant mutation.
This mutation caused an abnormal red blood
cell membrane (abnormal or lack of spectrin).
This disorder is most common in
people of Northern Europe
descent, but it has been found
in all races.
An 18-year old African American woman was
seen in the emergency department for fever and
abdominal pain. The following results were
obtained on a blood count:
i ã  count: 11.9 x 109/L - increased
i R  count: 3,67 x 1012/L - decreased
i Hgb: 10.9 g/dL decreased
i Hct: 32.5% (0.325L/L) decreased

i Platelet count : 410 x 109/L - normal


i RDã : 19.5% - increased
i Segmented neutrophils : 75% - normal
i Lymphocytes : 18% - normal
i Monocytes : 3% - normal
i Eosinophils : 3% - normal
i asophils : 1% - normal
i Reticulocyte count: 3.1% - increased
i
1.Select the confirmatory tests that should be
performed and the expected results.

- onfirmatory test should be performed are


hemoglobin solubility test and cirtate agar test,
hemoglobin   s separated from hemoglobin A, O, and
E as a result of mode of migration; hemoglobin 
migrates more toward the anode, whereas hemoglobins
A, O, and E migrate toward the cathode. Likewise,
hemoglobin S migrate toward the cathode. Likewise,
hemoglobin S migrates anodally, wheraes hemoglobins
D and G migrate cathodally.
2. Describe the characteristic R  morphology
on the peripheral blood film.

- haracteristically, red blood cell that contain


cystallized aggregates of hemoglobin that
protrude through the cell membrane are seen. They
are irregular shaped cells which appear to contain
mis-shapen crystals and increased target cells are
seen.
3. ased on the electrophoresis and R 
morphology results, what diagnosis is
suggested?

- On the basis of the ectrophoretic pattern,


the daignosis of presence of hemoglobin S
can be made
4. f this patient were to marry a person of genotype
HbAS, what would be the expected frequency of
genotypes for each of four children?

- According to Mendelian law, the genotype can be


depicted by the following chart:

A S

6 6 66

  6

þ  
 
i Mild, chronic hemolytic anemia associated with
variable vaso-occlusive complications.
i Splenomegaly

i Hgb level usually 11 Ȃ 13% g/dL


i Reticulocyte count: 3 Ȃ 5%
i Peripheral smear: few sickle cell, target cells. And intra
Ȃ erythrocyte free crystalline structures (may protrude
from membrane, HbS crystals)
i itrate agar:  migrates, separation from S, E, O
i Diagnostic Hematology by: Rodak, ernadette F.
i linical Hemetolology: orrelations and Principles by:
Steininger, Sheryl