The clinical pentad of thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare condition with variable clinical symptoms. The diagnosis of TTP should be considered in patients with unexplained neurological symptoms or acute renal insufficiency in combination with thrombocytopenia and anaemia. Its pathophysiology is based on autoantibodies which inhibit the cleaving of Von Willebrand Factor. This leads to thrombocytopenia and mico-angiopathic haemolytic anaemia. Without treatment TTP has a high mortality. The first line of treatment is plasma exchange, often combined with corticosteroids. If the patient is unresponsive to this then further treatment options are rituximab, other immune suppressants and as a last resort splenectomy can be considered. This report presents a patient with all the clinical characteristics of TTP, which is rarely seen these days.