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Dyskeratosis congenita (DC) is an inherited BMF syndrome characterized by the presence of short telomeres, mucocutaneous abnormalities, and cancer predisposition. While both conditions may cause BMF and hepatic impairment, therapeutic approaches are distinct, making it imperative to establish the correct diagnosis. In clinical practice, lymphocyte telomere lengths (TL) are used as a first\u2010line screen to rule out inherited telomeropathies before initiating treatment for AA. To evaluate the reliability of TL in the HAA population, we performed a retrospective analysis of TL in 10 consecutively enrolled HAA patients compared to 19 patients with idiopathic AA (IAA). HAA patients had significantly shorter telomeres than IAA patients (<jats:italic>P<\/jats:italic>\u2009=\u20090.009), including four patients with TL at or below the 1st percentile for age\u2010matched controls. HAA patients had no clinical features of DC and did not carry disease\u2010causing mutations in known genes associated with inherited telomere disorders. Instead, short TLs were significantly correlated with severe lymphopenia and skewed lymphocyte subsets, features characteristic of HAA. Our results indicate the importance of caution in the interpretation of TL measurements in HAA, because, in this patient population, short telomeres have limited specificity. Am. J. Hematol. 91:243\u2013247, 2016. \u00a9 2015 The Authors. American Journal of Hematology Published by Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/ajh.24256","type":"journal-article","created":{"date-parts":[[2015,11,30]],"date-time":"2015-11-30T10:00:40Z","timestamp":1448877640000},"page":"243-247","source":"Crossref","is-referenced-by-count":12,"title":["Disrupted lymphocyte homeostasis in hepatitis\u2010associated acquired aplastic anemia is associated with short telomeres"],"prefix":"10.1002","volume":"91","author":[{"given":"Daria V.","family":"Babushok","sequence":"first","affiliation":[{"name":"Comprehensive Bone Marrow Failure Center, Division of Hematology, Department of Pediatrics Children's Hospital of Philadelphia Philadelphia Pennsylvania"},{"name":"Division of Hematology\u2013Oncology, Department of Medicine Hospital of the University of Pennsylvania Philadelphia 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