Article
Paroxysmal nocturnal hemoglobinuria: current issues in pathophysiology and treatment.
Current hematology reports - 1 Mar 2005
Young Neal S
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired genetic disorder of the bone marrow that produces intravascular hemolysis, proclivity to venous thrombosis, and hematopoietic failure. Mutation in the PIG-A gene in a stem cell aborts synthesis of glycosyl-phosphoinositol (GPI) anchors and therefore expression of all GPI-anchored proteins on the surface of progeny erythrocytes, leukocytes, and platelets....
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