Paroxysmal nocturnal haemoglobinuria
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate system. This destructive process occurs due to of the red blood cell surface DAF, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood of the circulatory system, the red blood cell destruction (hemolysis) is considered an intravascular hemolytic . There is ongoing research into other key features of the disease, such as the high of blood clot formation. Research suggests that PNH is caused by both the absence of GPI-anchored complement regulatory proteins on PNH and the excessive consumption of nitric oxide (NO).
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