Diagnosis and management of thrombotic thrombocytopenic purpura (TTP) in Australia: Findings from the first 5 years of the Australian TTP/thrombotic microangiopathy registry

Abstract

Background: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy (TMA). In 2009, the Australian TTP/TMA registry was established to collect data on patients presenting with TTP/TMA throughout Australia. Aim: To summarise information on the diagnosis and management of patients with TTP collected in the first 5years (2009-2014) of the Australian TTP registry. Methods: Registry data from June 2009 to October 2014 were reviewed. Results: Fifty-seven patients were identified with TTP (defined as ADAMTS13 activity <10%), accounting for 72 clinical episodes. ADAMTS13 inhibitor testing was performed in nine out of 57 patients (16%), reflecting the limited ..