Paroxysmal Nocturnal Hemoglobinuria - PNH

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired clonal disorder caused by the nonmalignant clonal expansion of one or more stem cell lines. PNH results in a deficiency of cell-surface glycosylphosphatidylinositol (GPI)-anchored proteins, including complement pathway regulatory proteins. It is associated with several phenotypes or phenotype combinations, including intravascular hemolysis, thrombotic complication (especially Budd-Chiari syndrome and mesenteric thrombosis), and aplastic anemia (bone marrow failure).

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Last Update: May 2018