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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
- 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 01/2018