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Blood, 25 June 2009, Vol. 113, No. 26, pp. 6549-6557. Prepublished online as a Blood First Edition Paper on April 7, 2009; DOI 10.1182/blood-2008-12-192880. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2008-12-192880v1 blood-2008-12-192880v2 113/26/6549    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Google Scholar Articles by Alter, B. P. Articles by Rosenberg, P. S. PubMed PubMed Citation Articles by Alter, B. P. Articles by Rosenberg, P. S. Related Collections Hematopoiesis and Stem Cells Myeloid Neoplasia Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Cancer in dyskeratosis congenita Blanche P. Alter1, Neelam Giri1, Sharon A. Savage1, and Philip S. Rosenberg2 1 Clinical Genetics Branch and 2 Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Bethesda, MD Dyskeratosis congenita (DC) is a rare inherited bone marrow failure syndrome. The spectrum of cancer susceptibility in this disorder of telomere biology has not been described. There were more than 500 cases of DC reported in the literature from 1910 to 2008; the National Cancer Institute (NCI) prospective DC cohort enrolled 50 cases from 2002 to 2007. Sixty cancers were reported in 52 literature cases, while 7 occurred among patients in the NCI DC cohort. The 2 cohorts were comparable in their median overall survival (42 years) and cumulative incidence of cancer (40%-50% by age 50 years). The most frequent solid tumors were head and neck squamous cell carcinomas (40% of patients in either cohort), followed by skin and anorectal cancer. The ratio of observed to expected cancers (O/E ratio) in the NCI cohort was 11-fold compared with the general population (P < .05). Significantly elevated O/E ratios were 1154 for tongue cancer and 195 for acute myeloid leukemia. Survival after bone marrow transplantation for aplastic anemia or leukemia was poor in both cohorts. The frequency and types of cancer in DC are surpassed only by those in Fanconi anemia (FA), indicating that FA and DC have similarly high risks of adverse hematologic and neoplastic events, and patients with these diseases should be counseled and monitored similarly. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Cancer & inherited bone marrow failure states Kim E. Nichols and Monica Bessler Blood 2009 113: 6502-6503. [Full Text] [PDF] This article has been cited by other articles: Dyskeratosis Congenita and Cancer Journal Watch Oncology and Hematology, August 4, 2009; 2009(804): 1 - 1. [Full Text] K. E. Nichols and M. Bessler Cancer & inherited bone marrow failure states Blood, June 25, 2009; 113(26): 6502 - 6503. [Full Text] [PDF]

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