Clinical features

• Chronic intravascular haemolytic anaemia particularly overnight (?due to lower blood pH). Infections trigger acceleration of haemolysis.

• WBC and platelet production also often 5.

• Chronic haemolysis may induce nephropathy.

• Haemoglobinuria usually results in iron deficiency.

• 44 tendency to venous thrombosis particularly at atypical sites e.g. hepatic vein (Budd-Chiari syndrome), sagittal sinus thrombosis.

• Fatigue, dysphagia and impotence occasionally seen.

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