Thrombosis of Portal Vein in a Case with Paroxysmal Nocturnal Haemoglobinuria

Paroxysmal nocturnal haemoglobinuria is an acquired clonal stem cell disease, characterized by chronic intravascular haemolytic anemia, hypercoagulability, deficient haematopoiesis and rarely leukaemic conversion. The mechanism of haemolysis appears to be unregulated complement activation on the abnormal red cell surface, because of reduction or absence of regulatory membrane molecules protecting cells against the membrane attack complex of complement mediated lysis. Thrombosis in hepatic, portal, mesenteric, renal and cerebral veins (which has an incidence of 12-40 %) is one of the important complications of paroxysmal nocturnal haemoglobinuria.

Paroksismal Noktürnal Hemoglobinürili Bir Olguda Portal Ven Trombozu