The Progression of Study on the Pathogenesis of PNH Clone Extension

Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired hemolytic anemia that often manifests with a deficiency of glycosylphosphatidylinositol anchored proteins (GPI-AP) on the surface of the abnormal hematopoietic stem cells.The clinical manifestations of PNH are characterized by paroxysmal hemoglobinuria related to somnus,probably accompanied with the decreasing of the whole blood cells and iterative thrombosis.Usually,the complications of PNH are myelodysplastie syndrome and acute leukemia.Some researches confirm that the disease results from the expansion of hematopoietic stem cells harboring a mutation in PIG-A gene.Based on recent literatures,the pathogenesis of PNH clone extension are summarized in this review. Key words: Paroxysmal nocturnal hemoglobinuria; PIG-A gene mutation; PNH clone