Abstract
Paroxysmal nocturnal haemoglobinuria is a rare disease caused by relentless complement hyperactivation at the surface of erythrocytes that lack CD55 and CD59 regulatory proteins (paroxysmal nocturnal haemoglobinuria erythrocytes). CD59 is a terminal regulator of the C5 convertase, preventing the formation of the membrane attack complex, which is responsible for the characteristic intravascular haemolysis that causes most signs and symptoms of the disease. CD55 inhibits the C3 convertase, avoiding the accumulation of the opsonin C3b on the surface of erythrocytes and their phagocytosis by hepatosplenic macrophages.
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