Article
The pathophysiology of disease in patients with paroxysmal nocturnal hemoglobinuria.
Hematology. American Society of Hematology. Education Program - 1 Jan 2008
Bessler Monica, Hiken Jeffrey
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia caused by the expansion of a hematopoietic progenitor cell that has acquired a mutation in the X-linked PIGA gene. PNH occurs on the background of bone marrow failure. Bone marrow failure and the presence of the abnormal cells account for the clinical phenotype of patients with PNH including hemolysis, cytopenia, and thrombophilia. PIGA is...
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