1. Academic Validation
  2. Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia

Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia

  • Leukemia. 2007 Dec;21(12):2456-62. doi: 10.1038/sj.leu.2404968.
A Röth 1 J Dürig H Himmelreich S Bug R Siebert U Dührsen P M Lansdorp G M Baerlocher
Affiliations

Affiliation

  • 1 Department of Hematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany. alexander.roeth@uni-due.de
Abstract

To test the role of telomere biology in T-cell prolymphocytic leukemia (T-PLL), a rare aggressive disease characterized by the expansion of a T-cell clone derived from immuno-competent post-thymic T-lymphocytes, we analyzed telomere length and Telomerase activity in subsets of peripheral blood leukocytes from 11 newly diagnosed or relapsed patients with sporadic T-PLL. Telomere length values of the leukemic T cells (mean+/-s.d.: 1.53+/-0.65 kb) were all below the 1st percentile of telomere length values observed in T cells from healthy age-matched controls whereas telomere length of normal T- and B cells fell between the 1st and 99th percentile of the normal distribution. Leukemic T cells exhibited high levels of Telomerase and were sensitive to the Telomerase Inhibitor BIBR1532 at doses that showed no effect on normal, unstimulated T cells. Targeting the short telomeres and Telomerase activity in T-PLL seems an attractive strategy for the future treatment of this devastating disease.

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