MCQ – Haemoglobinopathy

Which of the following is least likely to have splenomegaly persisting to adulthood?

1. β⁺ thalassaemia intermedia
2. Sickle cell anaemia
3. Sickle cell trait
4. Sickle β⁺ thalassaemia

 

Which of the following is most commonly characterised by iron overload?

1. Alpha thalassaemia
2. β⁺ thalassaemia major
3. Sickle cell disease 
4. Hb D 

A Rh positive woman from South-east Asia delivers a neonate with hydrops fetalis. What is the likely cause?

1. α-thalassaemia
2. β-thalassaemia Major
3. δ-thalassaemia
4. Haemoglobin E diseases

Which of The following haemoglobinopathy does not give protection against malaria

1. Sickle cell anaemia
2. Haemoglobin C disease
3. Beta thalassaemia
4. Haemoglobin D

Which of the following statements about sickle cell anaemia is not true?

1. Hemoglobin S polymerises under hypoxic conditions
2. Hypoxia and dehydration increase the risk of sickling
3. Haemoglobin F increases the risk of sickling
4. Nigeria, democratic republic of Congo and India are the three countries with the highest number of patients with sickle cell anaemia

 

 

Answer 1

B. Sickle cell anaemia – Recurrent splenic infractions from early childhood result in a small fibrotic spleen

Answer 2

B. Patients with β⁺ thalassaemia major accumulate iron because of increased intestinal iron absorption and blood transfusion

Answer 3

A. α-thalassaemia. Barts hydrops faetalis results from a severe form of alpha thalassaemia resulting from the loss of all four alpha globin genes. This mainy seen in south east asia and Thailand.

Answer 4

D. Haemoglobin D does not protect against malaria, other haemoglobinopathies do

Answer 5

C. Haemoglobin F decreases the risk of sickling