A RARE CASE OF THROMBOTIC THROMBOCYTOPENIC PURPURA COMPLICATED BY MACROVASCULAR EVENTS

Abstract Thrombotic thrombocytopenic purpura is a rare but serious disease with high rates of mortality. It is a thrombotic microangiopathy associated with fever, thrombocytopenia, haemolytic anaemia and can also present with neurological and renal dysfunctions. It is caused by the reduced activity of ADAMTS13, a metalloprotease which cleaves von Willebrand factor resulting in widespread microvascular occlusion due to platelet rich thrombi. Although small vessel infarcts are common in kidney and brain, large vessel occlusions are rarely seen in thrombotic thrombocytopenic purpura. We present a case of a 56-year-old woman who presented with symptoms of urinary tract infection subsequently diagnosed with Thrombotic thrombocytopenic purpura. The initial treatment regimen consisted of plasma exchange therapy followed by steroids and cyclophosphamide. She was gradually improving, however her course of disease was complicated by a large cerebral infarct and bilateral pulmonary artery thrombosis resulting in intensive care unit admission and prolonged hospitalization. The patient was subsequently started on anti-coagulant therapy with fondaparinux and monitored continuously due to the increased risks of haemorrhage. The patient gradually improved over time with sustained improvement in laboratory reports. The outcomes of anti-coagulant therapy were favourable in our case and patient was discharged with rituximab therapy on subsequent follow ups. This case report intends to highlight the macrovascular thrombotic events and the challenge it brings to physicians regarding thrombolysis.