|
|
Prepublished online as a Blood First Edition Paper on May 22, 2003; DOI 10.1182/blood-2002-11-3444.
Submitted November 13, 2002
Accepted April 15, 2003
Refractory anemia in childhood: a retrospective analysis of 67 cases with particular reference to monosomy 7
Gabriela Kardos, Irith Baumann, S Jane Passmore, Franco Locatelli, Henrik Hasle, Kirk R Schultz, Jan Stary, Annette Schmitt-Graeff, Alexandra Fischer, Jochen Harbott, Judith M Chessells, Ian Hann, Susanna Fenu, Angelo Cantu Rajnoldi, Gitte Kerndrup, Elisabeth van Wering, Tim Rogge, Peter Noellke, and Charlotte M Niemeyer*
VU University Medical Center, Amsterdam, The Netherlands
Department of Pathology, University of Erlangen, Erlangen, Germany
Childhood Cancer Research Group, Oxford, United Kingdom
IRCCS Polioclinico S. Matteo, University of Pavia, Pavia, Italy
Skejby Hospital, University of Aarhus, Aarhus, Denmark
Department of Pediatrics, University of British Columbia, Vancouver, Canada
Department of Pediatrics, Charles University, Prague, Czech Republic
Department of Pediatrics and Pathology, University of Freiburg, Freiburg, Germany
Department of Pediatrics, University of Giessen, Giessen, Germany
Department of Haematology and Oncology, Great Ormond Street Children's Hospital, London, United Kingdom
Cattedra di Ematologia, University La Sapienza of Roma, Rome, Italy
Laboratory Department, Istituti Clinici di Perfezionamento, Milan, Italy
Department of Pathology, University of Odense, Odense, Denmark
Dutch Children Leukemia Study Group, The Hague, The Netherlands
* Corresponding author; email: niemeyer{at}kikli.ukl.uni-freiburg.de.
Primary myelodysplasia (MDS) without an increased number of blasts is a rare finding in childhood. We have performed a retrospective analysis of 67 children with a diagnosis of primary MDS to determine their clinical and hematological course. The median age at diagnosis was 8.3 years (range 0.3-18.1). In contrast to refractory anemia in adults, 44% of patients had hemoglobin greater than 10 g/100mL. The median white blood count and absolute neutrophil count were 3.6 and 0.9 x10 9/L, respectively. Seventy-five percent of patients were thrombocytopenic. The bone marrow was hypocellular in 43% of cases. The results of cytogenetic analysis showed monosomy 7 in 49% of patients, trisomy 8 in 9% and other abnormalities in 9%. The probability of survival 10 years after diagnosis was 0.48 (standard error [SE] = 0.10). Patients with monosomy 7 had a significantly higher estimated probability of progression to advanced MDS as compared to patients with other chromosomal anomalies or normal karyotype. Of the 67 children, 41 received an allogeneic stem cell transplant (SCT). Patients who had not progressed to advanced MDS prior to SCT had a significantly greater probability of survival than patients who suffered progression (0.76 [S.E. = 0.09] versus 0.36 [S.E. = 0.16]). SCT improved outcome 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 wait 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]
|
|
|
|
|
