Pathogenesis of paroxysmal nocturnal hemoglobinuria

Abstract excerpt

ABSTRACT: Paroxysmal nocturnal hemoglobinuria (PNH) is a nonmalignant clonal hematopoietic disorder. There are 2 components to the pathogenesis of PNH: (1) a mutant stem cell and (2) expansion of the mutant clone. Component 1 is straightforward: there is almost always an inactivating somatic mutation of the X-linked gene PIGA. As for component 2, different mechanisms may be involved. In rare cases, expansion may...

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