Tags: Disease.
Paroxysmal nocturnal hemoglobinuria (PNH) sometimes referred to as Marchiafava-Micheli syndrome is a rare genetically acquired life-threatening disease of the blood characterized by complement-induced intravascular hemolytic anemia (anemia due to destruction of red blood cells in the bloodstream) red urine (due to the appearance of hemoglobin in the urine) and thrombosis.PNH is the only hemolytic anemia which is most often caused by an acquired (rather than inherited) intrinsic defect in the cell membrane (deficiency of glycophosphatidylinositol leading to absence of protective proteins on the membrane).