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Prepublished online as a Blood First Edition Paper on May 22, 2003; DOI 10.1182/blood-2002-11-3444. This Article Full Text Full Text (PDF) All Versions of this Article: 2002-11-3444v1 102/6/1997    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 Kardos, G. Articles by Niemeyer, C. M. Search for Related Content PubMed PubMed Citation Articles by Kardos, G. Articles by Niemeyer, C. M. Related Collections Transplantation Clinical Trials and Observations Immunobiology Red Cells Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  Blood, 15 September 2003, Vol. 102, No. 6, pp. 1997-2003 CLINICAL OBSERVATIONS, INTERVENTIONS, AND THERAPEUTIC TRIALS Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7 Gabriela Kardos, Irith Baumann, S. Jane Passmore, Franco Locatelli, Henrik Hasle, Kirk R. Schultz, Jan Star, Annette Schmitt-Graeff, Alexandra Fischer, Jochen Harbott, Judith M. Chessells, Ian Hann, Susanna Fenu, Angelo Cantú Rajnoldi, Gitte Kerndrup, Elisabeth van Wering, Tim Rogge, Peter Nöllke, and Charlotte M. Niemeyer From the VU University Medical Center Amsterdam, The Netherlands; Department of Pathology, University of Erlangen, Germany; Childhood Cancer Research Group, Oxford, United Kingdom; IRCCS Policlinico S. Matteo, Pavia, Italy; Skejby Hospital, University of Aarhus, Denmark; Department of Pediatrics, University of British Columbia, Vancouver, Canada; Department of Pediatrics, Second Medical Faculty, Charles University, Prague, Czech Republic; Department of Pediatrics and Pathology, University of Freiburg, Germany; Department of Pediatrics, University of Giessen, Germany; Department of Haematology and Oncology, Great Ormond Street Children's Hospital, London, United Kingdom; Cattedra di Ematologia, University La Sapienza of Roma, Italy; Laboratory Department, Istituti Clinici di Perfezionamento, Milan, Italy; Department of Pathology, University of Odense, Denmark; and the Dutch Children Leukemia Study Group, The Hague, The Netherlands. Primary myelodysplasia (MDS) without an increased number of blasts is a rare finding in childhood. We performed a retrospective analysis of 67 children with a diagnosis of primary MDS to determine the clinical and hematologic course of the disease. The median age at diagnosis was 8.3 years (range, 0.3-18.1 years). In contrast to refractory anemia in adults, 44% of patients had hemoglobin levels greater than 10 g/100 mL. The median white blood cell count and the absolute neutrophil count were 3.6 x 109/L and 0.9 x 109/L, respectively. Seventy-five percent of patients had thrombocytopenia. Bone marrow was hypocellular in 43% of the patients. Results of cytogenetic analysis showed monosomy 7 in 49%, trisomy 8 in 9%, and other abnormalities in 9% of the patients. The probability of survival 10 years after diagnosis was 0.48 (standard error [SE] = 0.10). Patients with monosomy 7 had significantly higher estimated probabilities of progression to advanced MDS than did patients with other chromosomal anomalies or normal karyotype. Of the 67 children, 41 underwent allogeneic stem cell transplantation (SCT). Patients whose disease did not progress to advanced MDS before SCT had significantly greater probability of survival than patients who experienced progression (0.76 [SE = 0.09] vs 0.36 [SE = 0.16]). SCT improved the outcomes for patients with monosomy 7 and should be offered early in the course of the disease. Recommendations for best treatment options for children with other chromosomal abnormalities or normal karyotype may have to await results of prospective clinical trials. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: H. Hasle, T. A. Alonzo, A. Auvrignon, C. Behar, M. Chang, U. Creutzig, A. Fischer, E. Forestier, A. Fynn, O. A. Haas, et al. Monosomy 7 and deletion 7q in children and adolescents with acute myeloid leukemia: an international retrospective study Blood, June 1, 2007; 109(11): 4641 - 4647. [Abstract] [Full Text] [PDF] A. Yoshimi, I. Baumann, M. Fuhrer, E. Bergstrasser, U. Gobel, K.-W. Sykora, T. Klingebiel, U. Gross-Wieltsch, M. M van den Heuvel-Eibrink, A. Fischer, et al. Immunosuppressive therapy with anti-thymocyte globulin and cyclosporine A in selected children with hypoplastic refractory cytopenia Haematologica, March 1, 2007; 92(3): 397 - 400. [Abstract] [Full Text] [PDF] C.-H. Pui, M. Schrappe, R. C. Ribeiro, and C. M. Niemeyer Childhood and Adolescent Lymphoid and Myeloid Leukemia Hematology, January 1, 2004; 2004(1): 118 - 145. [Abstract] [Full Text] [PDF]

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