Article
[Advances in research for pathogenesis of paroxysmal nocturnal hemoglobinuria].
[Rinsho ketsueki] The Japanese journal of clinical hematology - 1 Jan 2021
Murakami Yoshiko
Abstract excerpt
Paroxysmal Nocturnal hemoglobinuria, PNH is usually caused by the somatic mutation of X-linked PIGA gene followed by the clonal expansion of the GPI (glycosylphosphatidylinositol) anchor defective hematopoietic stem cell clone. There are two hypotheses for the mechanism of clonal expansion, one is selection theory, in which GPI deficient cells escape from attacks of cytotoxic cells, and another is benign tumor...
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