clinical features

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Presentation is mainly in adults, with men affected more than women.

Clinical features may include:

  • paroxysmal nocturnal haemoglobinuria - present in only 25% of cases
  • severe haemolytic episodes may be associated with severe abdominal or back pain
  • chronic haemolysis is more common and often associated with iron deficiency
  • anaemia - variable; severe aplastic anaemia or acute haemolytic anaemia may occur
  • thromboses:
    • usually venous, often from atypical sites such as hepatic, portal and cerebral veins
    • arterial thromboses occur rarely
  • mild neutropenia and thrombocytopenia with infection

Last reviewed 05/2021