| Myelodysplastic and myeloproliferative disorders of childhood: a study of 167 patients. | |
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MedLine Citation:
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PMID: 9885207 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Myelodysplastic syndromes (MDS) and myeloproliferative syndromes (MPS) of childhood are a heterogeneous group of clonal disorders of hematopoiesis with overlapping clinical features and inconsistent nomenclature. Although a number of genetic conditions have been associated with MDS and MPS, the overall contribution of inherited predispositions is uncertain. We report a retrospective study examining clinical features, genetic associations, and outcomes in 167 children with MDS and MPS. Of these patients, 48 had an associated constitutional disorder. One hundred one patients had adult-type myelodysplastic syndrome (A-MDS), 60 had juvenile myelomonocytic leukemia (JMML), and 6 infants with Down syndrome had a transient myeloproliferative syndrome (TMS). JMML was characterized by young age at onset and prominent hepatosplenomegaly, whereas patients with A-MDS were older and had little or no organomegaly. The most common cytogenetic abnormalities were monosomy 7 or del(7q) (53 cases); this was common both in patients with JMML and those with A-MDS. Leukemic transformation was observed in 32% of patients, usually within 2 years of diagnosis. Survival was 25% at 16 years. Favorable prognostic features at diagnosis included age less than 2 years and a hemoglobin F level of less than 10%. Older patients tended to present with an adult-type MDS that is accommodated within the French-American-British system. In contrast, infants and young children typically developed unique disorders with overlapping features of MDS and MPS. Although the type and intensity of therapy varied markedly in this study, the overall outcome was poor except in patients with TMS. |
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Authors:
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S Luna-Fineman; K M Shannon; S K Atwater; J Davis; M Masterson; J Ortega; J Sanders; P Steinherz; V Weinberg; B J Lange |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Blood Volume: 93 ISSN: 0006-4971 ISO Abbreviation: Blood Publication Date: 1999 Jan |
Date Detail:
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Created Date: 1999-04-20 Completed Date: 1999-04-20 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 459-66 Citation Subset: AIM; IM |
Affiliation:
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University of California, San Francisco, San Francisco, CA 94143, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Child Child, Preschool Chromosome Aberrations Chromosomes, Human, Pair 7 Female Fetal Hemoglobin / metabolism Gene Deletion Humans Infant Infant, Newborn Leukemia / etiology Male Monosomy Myelodysplastic Syndromes* / diagnosis, genetics, therapy Myeloproliferative Disorders* / diagnosis, genetics, therapy Prognosis Retrospective Studies Survival Rate |
| Grant Support | |
ID/Acronym/Agency:
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CA13539/CA/NCI NIH HHS; M01-RR01271/RR/NCRR NIH HHS; R01-CA72614/CA/NCI NIH HHS |
| Chemical | |
Reg. No./Substance:
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9034-63-3/Fetal Hemoglobin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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