1999
DOI: 10.3109/10428199909058399
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Leukemia Arising out of Paroxysmal Nocturnal Hemoglobinuria

Abstract: In paroxysmal nocturnal hemoglobinuria (PNH), one or more hematopoietic stem cells that are defective in GPI anchor assembly as a result of mutation in the PIG-A gene preferentially expand in the bone marrow and give rise to peripheral blood elements that are deficient in GPI anchored protein expression. According to current concepts, 5-15% of PNH patients develop leukocyte dyscrasias which invariably are acute myelogenous leukemia (AML). In this review, the literature from 1962 to the present is analyzed rega… Show more

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Cited by 51 publications
(21 citation statements)
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“…Nocturnal hemoglobinuria is caused by a mutation at the PIG-A locus (Bessler et al 1994), and 5%-15% of such patients develop leukemia, invariably AML (Harris et al 1999). Underexpression of PIG-B would presumably result in low GPI anchor synthesis, and our results strongly suggest an important pharmacokinetic role for PIG-B.…”
Section: Pig-bmentioning
confidence: 99%
“…Nocturnal hemoglobinuria is caused by a mutation at the PIG-A locus (Bessler et al 1994), and 5%-15% of such patients develop leukemia, invariably AML (Harris et al 1999). Underexpression of PIG-B would presumably result in low GPI anchor synthesis, and our results strongly suggest an important pharmacokinetic role for PIG-B.…”
Section: Pig-bmentioning
confidence: 99%
“…[17][18][19] As in MDS, evolution of PNH to leukemia is possible (reviewed in Ref. 20), although with a much lower frequency. Conversely, MDS bears some similarity to AA and PNH in that some MDS patients will also respond to immunosuppression.…”
Section: Introductionmentioning
confidence: 99%
“…28 Above all, we have focused on the expansion of affected cells to manifest PNH symptoms. Increasing evidence suggests that the expansion is not simply attributable to PIGA mutations alone.…”
Section: Introductionmentioning
confidence: 99%