SOE 243: Thrombotic Thrombocytopenic Purpura (TTP)

Introduction A 36-year-old presents to hospital with a 2-week history of fever and feeling generally unwell. Initial blood tests show a platelet count of 28 x 109/L. The admitting consultant suspects...

What is thrombotic thrombocytopenic purpura (TTP)?

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy resulting in a consumptive thrombocytopenia, haemolytic anaemia and ischaemic end-organ damage It occurs due to a deficiency in the activity of a specific von Willebrand factor cleaving...

How common is TTP?

TTP is a rare condition: Prevalence is only 10-15 per 1,000,0000 Low incidence hinders the development of strong evidence base Has a female preponderance with a ratio of 2:1 Peak incidence is in adulthood before the age of...

What is the prognosis of patients with TTP?

Untreated the mortality is 90% The current mortality in the UK (from the TTP register) is 10-20%) Has significantly decreased since the introduction of early PEX Most deaths from cardiac and neurological complications Relapse rate is...

Which factors are associated with worse outcomes in TTP?

Age >60 Elevated troponin CNS involvement Delayed diagnosis Use of platelet transfusion Elevated LDH Refractory disease unresponsive to PLEX Episodes of relapse African or Caribbean ethnicity Consensus Guidelines G Related Pages: (5.8)...