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Blood, 1 January 2004, Vol. 103, No. 1, pp. 73-77. Prepublished online as a Blood First Edition Paper on August 28, 2003; DOI 10.1182/blood-2003-06-2146. This Article Full Text Full Text (PDF) All Versions of this Article: 2003-06-2146v1 103/1/73    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 Guardiola, P. Articles by Gluckman, E. Search for Related Content PubMed PubMed Citation Articles by Guardiola, P. Articles by Gluckman, E. Related Collections Immunobiology Transplantation Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Acute graft-versus-host disease in patients with Fanconi anemia or acquired aplastic anemia undergoing bone marrow transplantation from HLA-identical sibling donors: risk factors and influence on outcome Philippe Guardiola, Gérard Socié, Xiaxin Li, Patricia Ribaud, Agnès Devergie, Hélène Espérou, Patrice Richard, Richard Traineau, Anne Janin, and Eliane Gluckman From the Service d'Hématologie—Greffe de Moelle, "Trèfle 3," Hôpital Saint Louis, Paris, France; Etablissement Français du Sang, Hôpital Saint Louis, Paris, France; and Laboratoire de Pathologie ERM 02 20 INSERM, Université de Paris VII, Paris, France. To assess whether Fanconi anemia (FA) patients might be at risk for acute graft-versus-host disease (AGvHD) despite using low-intensity conditionings, we retrospectively analyzed the incidence of AGvHD and its impact on outcome in 37 FA patients and 73 patients with acquired aplastic anemia (AAA) that received transplants at Saint Louis Hospital from HLA-genotypic identical siblings with similar conditionings (thoraco-abdominal irradiation plus cyclophosphamide 20 [FA] or 150 mg/kg [AAA]). Despite being younger, FA patients had an increased risk of grades II to IV AGvHD (relative risk [RR], 2.00; P = .021), especially in younger patients (RR, 7.93; P = .014). The risks of requiring systemic corticosteroids to treat AGvHD and experiencing cortico-resistant AGvHD were significantly increased in FA patients. Although non-FA and FA patients had similar 10-year outcomes, acute and chronic GvHD had a biphasic effect on FA patient outcome with an additional cluster of lethal events starting by 5 years after transplantation. This late survival fall, restricted to FA patients, was closely related to head and neck carcinomas (15-year incidence: 53%). FA patients represent a group at risk regarding AGvHD when using irradiation-based conditionings. The impact of AGvHD on survival may not be limited to the early posttransplantation period and may be a major risk factor for head and neck carcinomas and late mortality in FA patients. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: B. Neven, S. Leroy, H. Decaluwe, F. Le Deist, C. Picard, D. Moshous, N. Mahlaoui, M. Debre, J.-L. Casanova, L. Dal Cortivo, et al. Long-term outcome after hematopoietic stem cell transplantation of a single-center cohort of 90 patients with severe combined immunodeficiency Blood, April 23, 2009; 113(17): 4114 - 4124. [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. Donor-derived oral squamous cell carcinoma after allogeneic bone marrow transplantation Blood, February 19, 2009; 113(8): 1834 - 1840. [Abstract] [Full Text] [PDF] M. W. Lensch and G. Q. Daley Scientific and clinical opportunities for modeling blood disorders with embryonic stem cells Blood, April 1, 2006; 107(7): 2605 - 2612. [Abstract] [Full Text] [PDF] E. C. Guinan Aplastic Anemia: Management of Pediatric Patients Hematology, January 1, 2005; 2005(1): 104 - 109. [Abstract] [Full Text] [PDF] P. S. Rosenberg, G. Socie, B. P. Alter, and E. Gluckman Risk of head and neck squamous cell cancer and death in patients with Fanconi anemia who did and did not receive transplants Blood, January 1, 2005; 105(1): 67 - 73. [Abstract] [Full Text] [PDF]

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