Severe telomere shortening in patients with paroxysmal nocturnal hemoglobinuria affects both GPI(-) and GPI(+) hematopoiesis Journal Article


Authors: Karadimitris, A.; Araten, D. J.; Luzzatto, L.; Notaro, R.
Article Title: Severe telomere shortening in patients with paroxysmal nocturnal hemoglobinuria affects both GPI(-) and GPI(+) hematopoiesis
Abstract: A most distinctive feature of paroxysmal nocturnal hemoglobinuria (PNH) is that in each patient glycosylphosphatidylinositol-negative (GPI(-)) and GPI(+) hematopoietic stem cells (HSCs) coexist, and both contribute to hematopoiesis. Telomere size correlates inversely with the cell division history of HSCs. In 10 patients with hemolytic PNH the telomeres in sorted GPI(-) granulocytes were shorter than in sorted GPI(+) granulocytes in 4 cases, comparable in 2 cases, and longer in the remaining 4 cases. Furthermore, the telomeres of both GPI(-) and GPI(+) hematopoietic cells were markedly shortened compared with age-matched controls. The short telomeres in the GPI(-) cells probably reflect the large number of cell divisions required for the progeny of a single cell to contribute a large proportion of hematopoiesis. The short telomeres of the GPI(+) cells indicate that the residual hematopoiesis contributed by these cells is not normal. This epigenetic change is an additional feature shared by PNH and aplastic anemia. (C) 2003 by The American Society of Hematology.
Keywords: age; dna; stem-cells; somatic mutations; insights; aplastic-anemia; pnh; pig-a; red cells
Journal Title: Blood
Volume: 102
Issue: 2
ISSN: 0006-4971
Publisher: American Society of Hematology  
Date Published: 2003-07-15
Start Page: 514
End Page: 516
Language: English
ACCESSION: WOS:000184083500023
DOI: 10.1182/blood-2003-01-0128
PROVIDER: wos
PUBMED: 12623835
Notes: Article; Proceedings Paper -- 41st Annual Meeting of the American-Society-of-Hematology -- DEC 03-08, 1999 -- NEW ORLEANS, LOUISIANA -- Source: Wos
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MSK Authors
  1. David Araten
    20 Araten
  2. Lucio Luzzatto
    104 Luzzatto