Article
[Paroxysmal nocturnal hemoglobinuria].
La Revue de medecine interne - 1 Mar 2010
Peffault de Latour R, Amoura Z, Socié G
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder of hematopoietic stem cells. PNH is related to a somatic mutation in the phosphatidylinositol glycan class A (PIG-A), X-linked gene, responsible for a deficiency in glycosylphosphatidylinositol-anchored proteins (GPI-AP). The lack of one of the GPI-AP complement regulatory proteins (CD59) leads to haemolysis. The disease is diagnosed with...
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