Decay-accelerating factor (CD55) deficiency

DAF deficiency is associated with Inab phenotype (absence of all blood group antigens of the Cromer complex). Clinically, PNH (paroxysmal nocturnal hemoglobinuria) usually affects young adults and has a variable course of intravascular haemolysis, pancytopenia, and recurrent (usually venous) thromboses. It often arises in patients with aplastic anaemia, and may transform into acute myeloblastic leukaemia. Complications include iron deficiency (from chronic haemoglobinuria), progressive renal impairment (from haemoglobinuria), and the Budd Chiari syndrome (hepatic vein thrombosis).

Therapeutic options