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Prepublished online as a Blood First Edition Paper on January 2, 2003; DOI 10.1182/blood-2002-10-3243. This Article Full Text (PDF) All Versions of this Article: 2002-10-3243v1 101/10/3872    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 Tonnies, H. Articles by Neitzel, H. Search for Related Content PubMed PubMed Citation Articles by Tonnies, H. Articles by Neitzel, H. Social Bookmarking                   What's this? Submitted October 25, 2002 Accepted December 27, 2002 Clonal chromosome aberrations in bone marrow cells of Fanconi anemia patients: gains of the chromosomal segment 3q26q29 as an adverse risk factor Holger Tonnies, Stefanie Huber, Jorn-Sven Kuhl, Antje Gerlach, Wolfram Ebell, and Heidemarie Neitzel* Institute of Human Genetics, Charite, Campus-Virchow, Berlin, Germany Department of General Pediatrics, Bone Marrow Transplant Unit, Charite, Campus-Virchow, Berlin, Germany * Corresponding author; email: Heidemarie.Neitzel{at}charite.de. Fanconi anemia (FA) is a condition with susceptibility to bone marrow failure, MDS, and leukemia. We report on a high incidence of expanding clonal aberrations with partial trisomies and tetrasomies of chromosome 3q in bone marrow cells of 18 out of 53 FA-patients analyzed, detected by conventional and molecular cytogenetics. To determine the clinical relevance of these findings, we compared the cytogenetic data, the morphologic features of the bone marrow, and the clinical course of these patients with 35 FA patients without clonal aberrations of 3q. Both groups did not differ significantly in respect to age, gender, and complementation group. There was a significant survival advantage of patients without abnormalities of chromosome 3q. Even more pronounced was the risk assessment of patients with gains of 3q material in respect to the development of morphologic MDS and AML. Thus, our data of 18 patients with 3q aberrations reveal that gains of 3q are strongly associated with a poor prognosis and represent an adverse risk factor in FA. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: B. P. Alter Diagnosis, Genetics, and Management of Inherited Bone Marrow Failure Syndromes Hematology, January 1, 2007; 2007(1): 29 - 39. [Abstract] [Full Text] [PDF] T. Taniguchi and A. D. D'Andrea Molecular pathogenesis of Fanconi anemia: recent progress Blood, June 1, 2006; 107(11): 4223 - 4233. [Abstract] [Full Text] [PDF] X. Li, M. M. Le Beau, S. Ciccone, F.-C. Yang, B. Freie, S. Chen, J. Yuan, P. Hong, A. Orazi, L. S. Haneline, et al. Ex vivo culture of Fancc-/- stem/progenitor cells predisposes cells to undergo apoptosis, and surviving stem/progenitor cells display cytogenetic abnormalities and an increased risk of malignancy Blood, May 1, 2005; 105(9): 3465 - 3471. [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]

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