Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH).

@article{Inoue2006MolecularBO,
  title={Molecular basis of clonal expansion of hematopoiesis in 2 patients with paroxysmal nocturnal hemoglobinuria (PNH).},
  author={Norimitsu Inoue and Tomohisa Izui-Sarumaru and Yoshiko Murakami and Yuichi Endo and Jun-ichi Nishimura and Ken Kurokawa and Maki Kuwayama and Hiroaki Shime and Takashi Machii and Yuzuru Kanakura and Gabrielle Meyers and Carl Wittwer and Zhong Chen and William Babcock and Debra A Frei-Lahr and Charles F. Parker and Taroh Kinoshita},
  journal={Blood},
  year={2006},
  volume={108 13},
  pages={4232-6}
}
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 apparent. Herein, we report 2 patients with PNH whose PIGA-mutant cells had a concurrent… CONTINUE READING