Article
Paroxysmal nocturnal hemoglobinuria: An acquired genetic disease.
American journal of hematology - 1 Nov 1999
Nishimura J, Murakami Y, Kinoshita T
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by an intravascular hemolytic anemia. Abnormal blood cells lack a series of glycosylphosphatidylinositol (GPI)-anchored proteins. The lack of GPI-anchored complement regulatory proteins, such as decay-accelerating factor (DAF) and CD59, results in complement-mediated hemolysis and hemoglobinuria. In the...
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