Topics In Pediatric Hematology Flashcards
Composition of HbA
α2β2 tetramer
Two alpha and two beta
subunits
Composition of HbF
α2γ2 tetramer
(Two alpha and two gamma
subunits.)
What is Transient Eryhtroblastopenia of Childhood(TEC)
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
Mode of inheritance of sickle cell
AR
What are the possibilities of children in a couple both carriers of an autosomal Recessive disorder?
25% normal
25% diseased
50% carriers
Management of sickle cell disease
Prophylaxis: Penicillin at age 3 m
Chronic blood transfusion + Desferroxamine or Exjade
Hydroxyurea: promotes HbF formation
BMT.
Blood smear of thalassemia shows….
microcytic red blood cells
anisopoikilocytosis with target cells
Transfusion dose for a pt. With thalassemia
15 ml/kg
What is the diagnostic limit for HbA2 in ana pt. With Thalasemia trait?
HbA2 >3.5%
How to Diagnose Thalasemia trait by electrophoresis?
HbA2 levels high
Management of thalassemia
• Transfusions
• Iron Chelation
• Hematopoietic Stem Cell Transplant
• Long-Term Monitoring for Complications includes:
- Hepatic and cardiac MRI for iron overload
- ECHO for cardiomyopathy
Pattern of inheritance of Hereditary spherocytosis
Autosomal Dominant
What is the cause of cell membrane defects in anemia?
Cytoskeleton proteins :Ankyrin, Band 3,
Protein 4, Actin and Spectrin
Lab Hallmarks of Fe deficiency anemia
Low Hb Low Hct Low Fe Low Ferritin High Transferrin/ TIBC
Treatment of Fe def. Anemia
Diet: Fe supplements(ferrous salts) Ferrous sulfate, Ferrous gluconate, Ferrous fumarate Sodium feredetate (Sytron) For at least 3-4 months