![]() 3 Multiple proposed mechanisms behind the increased incidence of thrombosis include a prothrombotic state in conjunction with platelet abnormalities and impaired fibrinolysis. 2 Despite such a large role in the burden of the disease, the mechanism behind the development of thrombosis is poorly understood, highlighting the importance of thrombosis management in PNH patients and elucidating more information regarding the nature of the thrombotic event. 1 Thromboembolic events are the most common cause of morbidity and mortality in PNH and account for 40–67% of deaths 40% of patients having suffered an event before diagnosis and 29–44% of patients suffering at least one event throughout the course of their disease. It is caused by an acquired mutation in the X-linked phosphatidylinositol glycan class A gene ( PIG-A), causing stem cell progeny (mature blood cells) to lack complement regulatory proteins and exposing them to complement attack. Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disorder of multipotent hematopoietic stem cells. Further studies, including novel in vivo and in vitro thrombosis models, are required in order to define the role of the individual mechanisms contributing to thrombosis, impaired fibrinolysis and clarify other complement-driven prothrombotic mechanisms in paroxysmal nocturnal hemoglobinuria. While many factors may affect thrombosis in paroxysmal nocturnal hemoglobinuria, the relative contribution of each mechanism that has been implicated is difficult to quantify. Impaired fibrinolysis has also been observed and may be caused by several mechanisms involving interactions between complement activation, coagulation and fibrinolysis. Platelet and endothelial microparticles as well as oxidative stress may play a role. ![]() Paroxysmal nocturnal hemoglobinuria leads to a complex and multifaceted prothrombotic state due to the pathological effects of platelet activation, intravascular hemolysis and neutrophil/monocyte activation. ![]() This review article discusses the different factors that contribute to the increased risk of thrombosis in paroxysmal nocturnal hemoglobinuria. The increased incidence of thrombosis in paroxysmal nocturnal hemoglobinuria is still poorly understood, but unlike many other thrombotic disorders, predominantly involves complement-mediated mechanisms. Paroxysmal nocturnal hemoglobinuria is a rare acquired hematologic disorder, the most serious complication of which is thrombosis.
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