Progressive Telomere Shortening in Aplastic Anemia
10.1182/blood.v91.10.3582
Crossref journal-article
American Society of Hematology
Blood (234)
Abstract

AbstractImproved survival in aplastic anemia (AA) has shown a high incidence of late clonal marrow disorders. To investigate whether accelerated senescence of hematopoietic stem cells might underlie the pathophysiology of myelodysplasia (MDS) or paroxysmal nocturnal hemoglobinuria (PNH) occurring as a late complication of AA, we studied mean telomere length (TRF) in peripheral blood leukocytes from 79 patients with AA, Fanconi anemia, or PNH in comparison with normal controls. TRF lengths in the patient group were significantly shorter for age than normals (P < .0001). Telomere shortening was apparent in both granulocyte and mononuclear cell fractions, suggesting loss at the level of the hematopoietic stem cell. In patients with acquired AA with persistent cytopenias (n = 40), there was significant correlation between telomere loss and disease duration (r = −.685; P < .0001), equivalent to progressive telomere erosion at 216 bp/yr, in addition to the normal age-related loss. In patients who had achieved normal full blood counts (n = 20), the rate of telomere loss had apparently stabilised. There was no apparent association between telomere loss and secondary PNH (n = 13). However, of the 5 patients in the study with TRF less than 5.0 kb, 3 had acquired cytogenetic abnormalities, suggesting that telomere erosion may be relevant to the pathogenesis of MDS in aplastic anemia.

Bibliography

Ball, S. E., Gibson, F. M., Rizzo, S., Tooze, J. A., Marsh, J. C. W., & Gordon-Smith, E. C. (1998). Progressive Telomere Shortening in Aplastic Anemia. Blood, 91(10), 3582–3592.

Authors 6
  1. Sarah E. Ball (first)
  2. Frances M. Gibson (additional)
  3. Siân Rizzo (additional)
  4. Jennifer A. Tooze (additional)
  5. Judith C.W. Marsh (additional)
  6. Edward C. Gordon-Smith (additional)
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Dates
Type When
Created 5 years, 10 months ago (Oct. 13, 2019, 10:40 p.m.)
Deposited 1 year, 1 month ago (July 24, 2024, 7:13 p.m.)
Indexed 1 week, 3 days ago (Aug. 23, 2025, 9:45 p.m.)
Issued 27 years, 3 months ago (May 15, 1998)
Published 27 years, 3 months ago (May 15, 1998)
Published Print 27 years, 3 months ago (May 15, 1998)
Funders 0

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@article{Ball_1998, title={Progressive Telomere Shortening in Aplastic Anemia}, volume={91}, ISSN={0006-4971}, url={http://dx.doi.org/10.1182/blood.v91.10.3582}, DOI={10.1182/blood.v91.10.3582}, number={10}, journal={Blood}, publisher={American Society of Hematology}, author={Ball, Sarah E. and Gibson, Frances M. and Rizzo, Siân and Tooze, Jennifer A. and Marsh, Judith C.W. and Gordon-Smith, Edward C.}, year={1998}, month=may, pages={3582–3592} }