Topics In Pediatric Hematology Flashcards

1
Q

Composition of HbA

A

α2β2 tetramer

Two alpha and two beta
subunits

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2
Q

Composition of HbF

A

α2γ2 tetramer
(Two alpha and two gamma
subunits.)

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3
Q

What is Transient Eryhtroblastopenia of Childhood(TEC)

A
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

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4
Q

Mode of inheritance of sickle cell

A

AR

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5
Q

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

A

25% normal
25% diseased
50% carriers

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6
Q

Management of sickle cell disease

A

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

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7
Q

Blood smear of thalassemia shows….

A

microcytic red blood cells

anisopoikilocytosis with target cells

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8
Q

Transfusion dose for a pt. With thalassemia

A

15 ml/kg

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9
Q

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

A

HbA2 >3.5%

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10
Q

How to Diagnose Thalasemia trait by electrophoresis?

A

HbA2 levels high

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11
Q

Management of thalassemia

A

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

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12
Q

Pattern of inheritance of Hereditary spherocytosis

A

Autosomal Dominant

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13
Q

What is the cause of cell membrane defects in anemia?

A

Cytoskeleton proteins :Ankyrin, Band 3,

Protein 4, Actin and Spectrin

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14
Q

Lab Hallmarks of Fe deficiency anemia

A
Low Hb
Low Hct
Low Fe
Low Ferritin
High Transferrin/ TIBC
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15
Q

Treatment of Fe def. Anemia

A
Diet:
Fe supplements(ferrous salts) 
Ferrous sulfate, Ferrous gluconate, Ferrous fumarate
Sodium feredetate (Sytron)
For at least 3-4 months
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16
Q

What is the cause of Iron resistant Iron deficiency anemia?

A

TMPRSS6 gene mutation

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