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Thrombotic Thrombocytopenia Purpura (Causes (Cancer, Pregnancy, Autoimmune…
Thrombotic Thrombocytopenia Purpura
Key Facts
Widespread adhesion and aggregation of platelets leads to microvascular thrombosis and thus consumption of platelets and thus profound thrombocytopenia
Causes
Cancer
Pregnancy
Autoimmune e.g. SLE
Drug associated
Idiopathic
Clinical Presentation
Fever
Fluctuating cerebral dysfunction
Florid purpura
Haemolytic anaemia with red cell fragmentation, often accompanied by AKI
Diagnosis
Coagulation screen is normal
Lactate dehydrogenase is raised as result of haemolysis
Treatment
IV METHYLPREDNISOLONE
IV RITUXIMAB
Plasma exchange to remove antibody to ADAMTS-13 as well as provide a source of ADAMTS-13