Document Detail


Refractory anemia in childhood: a retrospective analysis of 67 patients with particular reference to monosomy 7.
MedLine Citation:
PMID:  12763938     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Gabriela Kardos; Irith Baumann; S Jane Passmore; Franco Locatelli; Henrik Hasle; Kirk R Schultz; Jan Starý; 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 Nollke; Charlotte M Niemeyer
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2003-05-22
Journal Detail:
Title:  Blood     Volume:  102     ISSN:  0006-4971     ISO Abbreviation:  Blood     Publication Date:  2003 Sep 
Date Detail:
Created Date:  2003-09-08     Completed Date:  2003-10-17     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7603509     Medline TA:  Blood     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1997-2003     Citation Subset:  AIM; IM    
Affiliation:
Division of Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Mathildenstrasse 1, 79106 Freiburg, Germany. niemeyer@kikli.ukl.uni-freiburg.de
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Anemia, Refractory / genetics*,  mortality*,  therapy
Child
Child, Preschool
Chromosomes, Human, Pair 6
Chromosomes, Human, Pair 7*
Chromosomes, Human, Pair 8
Disease Progression
Female
Humans
Infant
Karyotyping
Male
Monosomy*
Neutropenia / genetics,  mortality
Prognosis
Retrospective Studies
Stem Cell Transplantation
Trisomy

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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