Article
Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH).
Blood - 15 Dec 2006
Inoue Norimitsu, Izui-Sarumaru Tomohisa, Murakami Yoshiko, Endo Yuichi, Nishimura Jun-Ichi, Kurokawa Ken, Kuwayama Maki, Shime Hiroaki, Machii Takashi, Kanakura Yuzuru, Meyers Gabrielle, Wittwer Carl, Chen Zhong, Babcock William, Frei-Lahr Debra, Parker Charles J, Kinoshita Taroh
Abstract excerpt
Somatic mutation of PIGA in hematopoietic stem cells causes deficiency of glycosyl phosphatidylinositol-anchored proteins in paroxysmal nocturnal hemoglobinuria (PNH) that underlies the intravascular hemolysis but does not account for expansion of the PNH clone. Immune mechanisms may mediate clonal selection but appear insufficient to account for the clonal dominance necessary for PNH to become clinically...
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