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Blood, 1 March 2007, Vol. 109, No. 5, pp. 2256-2262. Prepublished online as a Blood First Edition Paper on October 12, 2006; DOI 10.1182/blood-2006-07-036657. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2006-07-036657v1 109/5/2256    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 Citing Articles via Google Scholar Google Scholar Articles by Wagner, J. E. Articles by Auerbach, A. D. Search for Related Content PubMed PubMed Citation Articles by Wagner, J. E. Articles by Auerbach, A. D. Related Collections Hematopoiesis and Stem Cells Red Cells Transplantation Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  TRANSPLANTATION Unrelated donor bone marrow transplantation for the treatment of Fanconi anemia John E. Wagner1, Mary Eapen2, Margaret L. MacMillan1, Richard E. Harris3, Ricardo Pasquini4, Farid Boulad5, Mei-Jie Zhang2, and Arleen D. Auerbach6 1 Division of Pediatric Hematology, Oncology, and Blood and Marrow Transplantation, University of Minnesota Blood and Marrow Transplant Program, Minneapolis; 2 Center for International Blood and Marrow Transplant Research, Medical College of Wisconsin, Milwaukee; 3 Cincinnati Children's Hospital, OH; 4 Hospital de Clinicas–Federal University of Parana, Brazil; 5 Memorial Sloan-Kettering Cancer Center, New York, NY; 6 Laboratory of Human Genetics and Hematology, Rockefeller University, New York, NY Bone marrow transplantation (BMT) is the only known cure for the hematologic manifestations of Fanconi anemia (FA). Potential benefits of unrelated donor BMT for FA, however, have been severely limited by graft rejection and treatment-related mortality with resultant poor survival. Therefore, we evaluated the impact of potential prognostic factors on hematopoietic recovery, graft-versus-host disease (GVHD), and mortality in 98 recipients of unrelated donor BMT who received transplants between 1990 and 2003. Probabilities of neutrophil (89% vs 69%; P = .02) and platelet (74% vs 23%; P < .001) recovery were higher after fludarabine-containing regimens than nonfludarabine-containing regimens. Risks of acute GVHD (relative risk [RR], 4.29; P < .001) were higher with non–T-cell–depleted grafts. The day-100 mortality rate was significantly higher after nonfludarabine-containing regimens than fludarabine-containing regimens (65% vs 24%, respectively; P < .001). Corresponding 3-year adjusted overall survival rates were 13% versus 52% (P < .001). In addition, mortality was higher in recipients who were older (> 10 years), who were cytomegalovirus (CMV) seropositive, and who received more than 20 blood product transfusions before BMT. Based on these results, significant practice changes are suggested: use of a fludarabine-containing conditioning regimen in the context of T-cell–depleted marrow allografts, and earlier referral for transplantation prior to excessive transfusions in patients with marrow failure. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: M. D. Milsom, A. W. Lee, Y. Zheng, and J. A. Cancelas Fanca-/- hematopoietic stem cells demonstrate a mobilization defect which can be overcome by administration of the Rac inhibitor NSC23766 Haematologica, July 1, 2009; 94(7): 1011 - 1015. [Abstract] [Full Text] [PDF] 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] Y. Si, A. C. Pulliam, Y. Linka, S. Ciccone, C. Leurs, J. Yuan, O. Eckermann, S. Fruehauf, S. Mooney, H. Hanenberg, et al. 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