thrombotic thrombocytopenic purpura

Pathogenesis of thrombotic thrombocytopenic purpura (TTP)
abnormally large and sticky multimers of von Willebrand's factor cause platelets to clump within vessels
in TTP there is a deficiency of protease which breakdowns large multimers of von Willebrand's factor
overlaps with haemolytic uraemic syndrome (HUS)

Thrombotic thrombocytopenic purpura (TTP) is classically characterised as a pentad of: thrombocytopenia, microvascular haemolysis, fluctuating neurological signs, renal impairment and fever.

TTP has an untreated mortality of up to 90% and therefore rapid plasma exchange (PEX) may be a life saving intervention. Platelet transfusion in TTP is only indicated if there is an on-going life-threatening bleed. Intravenous methylprednisolone is indicated after treatment with PEX has been completed.

no antibiotics - may worsen outcome
plasma exchange is the treatment of choice
steroids, immunosuppressants

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