W33/L7: Thalassaemia Flashcards
1
Q
What’s the term for normal adult haemoglobin?
A
HbA
2
Q
What are the components of HbA?
A
A tetramer: α2β2
3
Q
What is foetal Hb composed of?
A
α2γ2
4
Q
Why does the fetus have different Hb?
A
HbF has high affinity for oxygen uptake, so helps with fetal oxygenation
5
Q
What’s deficient in α-thalassaemia?
A
α-globulin chains
6
Q
What haemoglobin structures do people with α-thalassaemia have?
A
γ4 or β4
7
Q
What’s deficient in β-thalassaemia?
A
β-globulin chains
8
Q
What Hb structures do people with β-thalassaemia have?
A
α4
9
Q
What’s the other name for homozygous β-thalassaemia?
A
Thalassaemia major
10
Q
What’s the clinical consequence of β-thalassaemia?
A
Haemolytic anaemia
11
Q
How does β-thalassaemia lead to haemolytic anaemia?
A
The α4 form aggregates, accumulates and precipitates in the RBCs causing damage
