Clinical haematology · UKMLA & AKT
Thrombotic thrombocytopenic purpura (TTP)
A free high-yield preview for the UKMLA Applied Knowledge Test. Below are the key points to recognise thrombotic thrombocytopenic purpura (ttp) — the full SA Note notes add investigations, management, complications and 10 practice questions.
Key high-yield points
- TTP is a thrombotic microangiopathy caused by absent/severely deficient ADAMTS13, leading to accumulation of ultra-large vWF multimers, platelet-rich microthrombi, and mechanical red cell fragmentation (microangiopathic haemolytic anaemia, MAHA).
- Acquired TTP - IgG autoantibodies against ADAMTS13 (most common); triggers include pregnancy, HIV, SLE, drugs
- Congenital TTP (Upshaw-Schulman syndrome) - ADAMTS13 gene mutations; presents in childhood
- Drugs - quinine (most classic), ciclosporin, ticlopidine, mitomycin C, tacrolimus
- Overrepresentation of Black African and Afro-Caribbean individuals in TTP registries
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