Thrombotic Thrombocytopenic Purpura

Rare form of thrombotic microangiopathy characterised by microangiopathic haemolysis, thrombocytopenia, and neurological abnomalities

Aetiology

  • Due to a deficiency of metalloproteinase ADAMTS13
  • Associated with triggers such as medication, AIDS and malignancy

Clinical presentation

  • Characterized by a classic pentad of fever, haemolytic anaemia, thrombocytopenia, acute renal failure, and neurological symptoms

Investigations

  • Blood film - schistocytes (consistent with haemolysis), reticulocyte count is elevated
  • Other bloods - creatinine level may be elevated, LDH level is extremely elevated, indirect bilirubin level is elevated
  • Urinalysis - proteinuria and microscopic haematuria

Management

  • IV plasma exchange
  • Splenectomy may be used to treat patients who do not respond to plasma exchange, or who relapse chronically