| 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 |
Related Documents
:
|
2178458 - Acute megakaryoblastic leukemia in children identified by immunological marker studies. 10475618 - The prognostic significance of 13q14 deletions in chronic lymphocytic leukemia. 18351338 - Therapy-related patterns of cytogenetic abnormalities in acute myeloid leukemia and mye... 10848828 - Signal antonymy unique to myelodysplastic marrows correlates with altered expression of... 21618718 - Pulmonary maturational arrest and death in a patient with pulmonary interstitial glycog... 19435438 - Sensitivity of mouse bioassay in clinical wound botulism. |
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 |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| 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
Previous Document: Selective depletion of donor alloreactive T cells without loss of antiviral or antileukemic response...
Next Document: Safety and effectiveness of long-term therapy with the oral iron chelator deferiprone.