Paroxysmal Nocturnal Hemoglobinuria (PNH): A progressive, life-threatening disease that causes thrombosis, end organ damage and impaired quality of life1-3
PNH Red Blood Cell (RBC)
PNH RBC Lysis (Hemolysis)
PNH is an acquired disease that leads to the hemolysis of red blood cells due to uncontrolled complement activation1,2,5
PNH is caused by an acquired somatic mutation of the PIG-A gene which leads to a lack of glycosylphosphatidylinositol-linked surface proteins, including the complement regulatory proteins CD55 and CD59
RBCs deficient in CD55 and CD59 are susceptible to chronic intravascular complement-mediated hemolysis
Hemolysis leads to the release of hemoglobin and nitric oxide scavenging, both of which are involved in the underlying consequences of PNH
Chronic complement-mediated hemolysis is the underlying cause of progressive morbidities and mortality in PNH
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