All::Haematology::Diseases::Thrombotic thrombocytopenic purpura

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What patient groups is Thrombotic thrombocytopenic purpura most common?

rare, typically adult females

Thrombotic thrombocytopenic purpura

What are the main clinical features of Thrombotic thrombocytopenic purpura?

  • fever
  • fluctuating neuro signs (microemboli)
  • microangiopathic haemolytic anaemia
  • thrombocytopenia
  • renal failure

Thrombotic thrombocytopenic purpura

What is the management of Thrombotic thrombocytopenic purpura?

  • no antibiotics - may worsen outcome
  • plasma exchange is the treatment of choice
  • steroids, immunosuppressants
  • vincristine

Thrombotic thrombocytopenic purpura

What are the main causes of Thrombotic thrombocytopenic purpura?

  • post-infection e.g. urinary, gastrointestinal
  • pregnancy
  • drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel, aciclovir
  • tumours
  • SLE
  • HIV

Thrombotic thrombocytopenic purpura

What is the pathophysiology of Thrombotic thrombocytopenic purpura?

  • abnormally large and sticky multimers of von Willebrand's factor cause platelets to clump within vessels
  • caused by a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns ('cleaves') large multimers of von Willebrand's factor

Thrombotic thrombocytopenic purpura