Clinical haematology · UKMLA & AKT
Sickle cell disease
A free high-yield preview for the UKMLA Applied Knowledge Test. Below are the key points to recognise sickle cell disease — the full SA Note notes add investigations, management, complications and 10 practice questions.
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
Unlock the full Sickle cell disease revision
Get the complete high-yield notes (4 more sections covering investigations, management and complications), 10 practice questions, mock exams and AI tutoring. Start free.