clinical features

Last reviewed 05/2021

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