Clinical haematology · UKMLA & AKT
Sickle cell disease
A free high-yield preview for the UKMLA Applied Knowledge Test, covering how to recognise this condition. The full SA Note notes add investigations, management and complications, with practice questions on this topic.
Key high-yield points
- Single point mutation in HBB gene: glutamate → valine at codon 6, producing HbS
- Deoxygenated HbS polymerises → rigid sickle cells → microvascular occlusion + chronic haemolysis
- Red cell lifespan 10-20 days (normal 120 days) → chronic haemolytic anaemia with high reticulocyte count
- Repeated splenic infarctions → autosplenectomy → functional hyposplenism → Howell-Jolly bodies persist on blood film
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