Topics In Pediatric Hematology

Question 1

Composition of HbA

Answer 1

α2β2 tetramer

Two alpha and two beta
subunits

Question 2

Composition of HbF

Answer 2

α2γ2 tetramer
(Two alpha and two gamma
subunits.)

Question 3

What is Transient Eryhtroblastopenia of Childhood(TEC)

Answer 3

A form of pure red cell aplasia (anemia)
• Benign self limiting condition
• Peak incidence: 2-3 years
• It can occur in infancy
• Immune mediated like Immune thrombocytopenia ITP
• Full haematological recovery

DD: diamond blackfan anemia, parvovirus B19

Question 4

Mode of inheritance of sickle cell

Answer 4

AR

Question 5

What are the possibilities of children in a couple both carriers of an autosomal Recessive disorder?

Answer 5

25% normal
25% diseased
50% carriers

Question 6

Management of sickle cell disease

Answer 6

Prophylaxis: Penicillin at age 3 m
Chronic blood transfusion + Desferroxamine or Exjade
Hydroxyurea: promotes HbF formation
BMT.

Question 7

Blood smear of thalassemia shows….

Answer 7

microcytic red blood cells

anisopoikilocytosis with target cells

Question 8

Transfusion dose for a pt. With thalassemia

Answer 8

15 ml/kg

Question 9

What is the diagnostic limit for HbA2 in ana pt. With Thalasemia trait?

Answer 9

HbA2 >3.5%

Question 10

How to Diagnose Thalasemia trait by electrophoresis?

Answer 10

HbA2 levels high

Question 11

Management of thalassemia

Answer 11

• Transfusions
• Iron Chelation
• Hematopoietic Stem Cell Transplant
• Long-Term Monitoring for Complications includes:
- Hepatic and cardiac MRI for iron overload
- ECHO for cardiomyopathy

Question 12

Pattern of inheritance of Hereditary spherocytosis

Answer 12

Autosomal Dominant

Question 13

What is the cause of cell membrane defects in anemia?

Answer 13

Cytoskeleton proteins :Ankyrin, Band 3,

Protein 4, Actin and Spectrin

Question 14

Lab Hallmarks of Fe deficiency anemia

Answer 14

Low Hb
Low Hct
Low Fe
Low Ferritin
High Transferrin/ TIBC

Question 15

Treatment of Fe def. Anemia

Answer 15

Diet:
Fe supplements(ferrous salts) 
Ferrous sulfate, Ferrous gluconate, Ferrous fumarate
Sodium feredetate (Sytron)
For at least 3-4 months

Question 16

What is the cause of Iron resistant Iron deficiency anemia?

Answer 16

TMPRSS6 gene mutation

This gene provides instructions for making a protein
called matriptase-2, which helps regulate iron levels
in the body