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Blood, 1 January 2005, Vol. 105, No. 1, pp. 67-73. Prepublished online as a Blood First Edition Paper on August 26, 2004; DOI 10.1182/blood-2004-04-1652. This Article Full Text (PDF) All Versions of this Article: 2004-04-1652v1 105/1/67    most recent Alert me when this article is cited Alert me if a correction is posted 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 Citing Articles via Google Scholar Google Scholar Articles by Rosenberg, P. S Articles by Gluckman, E. Search for Related Content PubMed PubMed Citation Articles by Rosenberg, P. S Articles by Gluckman, E. Social Bookmarking                   What's this?  Previous Article  |  Next Article  Submitted April 29, 2004 Accepted August 5, 2004 Risk of Head and Neck Squamous Cell Cancer and Death in Transplanted and Untransplanted Patients with Fanconi Anemia Philip S Rosenberg*, Gerard Socie, Blanche P Alter, and Eliane Gluckman Biostatistics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, MD, USA Service d'Hematologie/Greffe de Moelle, Hopital Saint Louis, Paris, France Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, MD, none * Corresponding author; email: rosenbep{at}mail.nih.gov. Hematopoietic stem cell transplant (SCT) is currently the only therapy that can restore normal hematopoiesis in patients with Fanconi Anemia (FA). FA patients have a high baseline risk of squamous cell cancers of the head, neck, and esophagus (SCC), and SCT conditioning may increase SCC incidence. We evaluated the risks of SCC and death in 145 untransplanted FA patients in the North American Survey (NAS) cohort, and 117 transplanted FA patients in the Hopital Saint Louis (SLH) cohort. The age-specific hazard of SCC was 4.4-fold higher in transplanted versus untransplanted FA patients (P = 0.003), and SCC occurred at significantly younger ages in the former (respective medians: 18 and 33 years, P = 0.004). Survival after SCC was similarly poor in both cohorts (P = 0.135, median = 13 months). The hazard of SCC increased at a greater than linear rate, to 4.4%/y by age 40 in NAS and 4.7%/y by 10 years after transplant in SLH. In SLH, the hazard of non-SCC death was biphasic, declining significantly (P = 0.004) from 7.1%/month during the first six months after transplant to 0.13%/month (1.6%/y) after the first year. Acute and chronic graft versus host diseases were significant SCC risk factors. Adverse event rates in these cohorts provide historical control rates to assess emerging therapies for FA. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: B. P. Alter, N. Giri, S. A. Savage, and P. S. Rosenberg Cancer in dyskeratosis congenita Blood, June 25, 2009; 113(26): 6549 - 6557. [Abstract] [Full Text] [PDF] F. O. Pinto, T. Leblanc, D. Chamousset, G. Le Roux, B. Brethon, B. Cassinat, J. Larghero, J.-P. de Villartay, D. Stoppa-Lyonnet, A. Baruchel, et al. Diagnosis of Fanconi anemia in patients with bone marrow failure Haematologica, April 1, 2009; 94(4): 487 - 495. [Abstract] [Full Text] [PDF] A. Janin, H. Murata, C. Leboeuf, J.-M. Cayuela, E. Gluckman, L. Legres, A. Desveaux, M. Varna, P. Ratajczak, J. Soulier, et al. 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