Haematology 16: Paediatric Haematology

Question 1

What is Transient abnormal myelopoiesis ?

Answer 1

• Congenital form of leukaemia commonly affecting children with Down syndrome.
• Tends to remit within the first 2 months of life
• Mainly affecting megakaryocyte lineage

Question 2

On which chromosome is the ß cluster ?

Answer 2

Chromosome 11

Question 3

On which chromosome is the å cluster ?

Answer 3

Chromosome 16

Question 4

Which Globin chains make up HbA ?

Answer 4

å2ß2

Question 5

Which Globin chains make up HbA2 ?

Answer 5

å2∂2 (alpha and delta)

Question 6

Which Globin chains make up HbF ?

Answer 6

alpha 2 gamma 2

Question 7

Why does Sickle cell disease not present in the first 6 months of life ?

Answer 7

HbF is still in high concentration and is able to compensate

Question 8

Name the test used to diagnose sickle cell anaemia at birth ?

Answer 8

The Guthrie spot test

Question 9

Why do children with sickle cell experience hand-foot syndrome whereas adults get bone pain in the central skeleton?

Answer 9

• Red bone marrow (where red blood cells and leukocytes are produced) is only present in the central skeleton in adults but in all bones in children.
• Sickle cells cause infarction of the vessels that supply the bone marrow causing pain

Question 10

What happens if a child with sickle cell anaemia gets a parvovirus b19 infection ?

Answer 10

Aplastic anaemia

Question 11

What are some of the clinical features of thalassaemia Major ?

Answer 11

Bossing of facial bones
Hepatosplenomegaly
Iron overload- heart/liver failure
Growth retardation

Question 12

What is the main treatment for vWD ?

Answer 12

Factor 8 concentrates

Question 13

What is the most common leukaemia of childhood ?

Answer 13

ALL