Name: Foad Mohamed                          

Theme: Paediatric haematology

 

OPTION LIST

 

A

Acute myeloid leukaemia

I

Immune thrombocytopenic purpura

B

Acute lymphocytic leukaemia

J

Sickle cell anaemia

C

Autoimmune haemolytic anaemia (AIHA)

K

Thrombocytopenia-absent radius syndrome

 

D

Beta thalassaema major

L

Transient abnormal myelopoiesis (TAM)

E

Evan’s Syndrome

M

 

F

Glucose 6-phosphate dehydrogenase deficiency

N

 

G

Haemophilia A

O

 

H

Haemophilia B

P

 

 

 

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

 

1. A rare myelodysplastic syndrome associated primarily with trisomy 21 with prominent involvement of megakaryocytes. Neonates often die from infiltration of leukaemic cells causing liver failure or hyper-viscosity. However, the disease process usually undergoes spontaneous remission with only 20 – 30% of affected infants dying without treatment

 

 

2. Clinical features of this haematological disorder become manifest in the child due to the decrease in gamma chain production and haemoglobin F synthesis after birth with concurrent increase in the synthesis of betaS and haemoglobin S

 

 

3. An 8 year old boy from the island of St. Kitts presents with sudden onset severe abdominal pain, vomiting and jaundice. A full blood count shows normal white blood count, platelets and MCV but reveals marked anaemia with raised unconjugated bilirubin. Blood film reveals irregularly contracted red cells and polychromatic macrocytes

 

 

4. A 2 year old boy presents with monoarticular joint pain and swelling of the knee which is revealed to be haemarthrosis. Investigations show normal FBC with prolonged aPTT and bleeding time and normal PT

 

 

5. A pale 7 year old boy was brought to A&E by a distressed mother with acute jaundice, widespread petechiae and ecchymoses. Full blood counts revealed a dramatic thrombocytopenia with severe anaemia. Further tests reveal reduced serum IgG, IgM and IgA with a positive Coomb’s test.

 

 

 

ANSWERS

1. L

2. J

3. F

4. G

5. E