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Article: Familial acute myeloid leukemia with monosomy 7: Late onset and involvement of a multipotential progenitor cell

TitleFamilial acute myeloid leukemia with monosomy 7: Late onset and involvement of a multipotential progenitor cell
Authors
Issue Date2000
PublisherElsevier Inc. The Journal's web site is located at http://www.elsevier.com/locate/cancergene
Citation
Cancer Genetics And Cytogenetics, 2000, v. 116 n. 2, p. 170-173 How to Cite?
AbstractFamilial acute myeloid leukemia (AML) with monosomy 7 is a rare syndrome with fewer than 10 families reported. The salient features included a young median age (8 years) at presentation, equal sex preference, and occurrence of cytopenias and myelodysplasia in nonleukemic family members. Owing to its rarity and the fact that many cases were reported quite some time ago, detailed clinicopathologic features of familial monosomy 7 were not available. We describe a family with three siblings affected by AML in whom monosomy 7 was demonstrated. This family showed several unique features, including the late onset of AML (at 34 and 37 years of age in two siblings), and the presence of an antecedent myelodysplastic phase before leukemia development. With fluorescence in situ hybridization, the monosomy 7 clone was shown to be capable of partial maturation, which was consistent with the biologic behavior of myelodysplasia. These observations suggest that familial leukemia with monosomy 7 is probably a multistep leukemogenic process in which monosomy 7 might be but one of the critical steps. Finally, the prognosis in these cases was poor, suggesting that more aggressive therapy may be needed to improve treatment outcome. Copyright (C) 2000 Elsevier Science Inc.
Persistent Identifierhttp://hdl.handle.net/10722/78198
ISSN
2012 Impact Factor: 1.929
2013 SCImago Journal Rankings: 0.872
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorKwong, YLen_HK
dc.contributor.authorNg, MHen_HK
dc.contributor.authorMa, SKen_HK
dc.date.accessioned2010-09-06T07:40:14Z-
dc.date.available2010-09-06T07:40:14Z-
dc.date.issued2000en_HK
dc.identifier.citationCancer Genetics And Cytogenetics, 2000, v. 116 n. 2, p. 170-173en_HK
dc.identifier.issn0165-4608en_HK
dc.identifier.urihttp://hdl.handle.net/10722/78198-
dc.description.abstractFamilial acute myeloid leukemia (AML) with monosomy 7 is a rare syndrome with fewer than 10 families reported. The salient features included a young median age (8 years) at presentation, equal sex preference, and occurrence of cytopenias and myelodysplasia in nonleukemic family members. Owing to its rarity and the fact that many cases were reported quite some time ago, detailed clinicopathologic features of familial monosomy 7 were not available. We describe a family with three siblings affected by AML in whom monosomy 7 was demonstrated. This family showed several unique features, including the late onset of AML (at 34 and 37 years of age in two siblings), and the presence of an antecedent myelodysplastic phase before leukemia development. With fluorescence in situ hybridization, the monosomy 7 clone was shown to be capable of partial maturation, which was consistent with the biologic behavior of myelodysplasia. These observations suggest that familial leukemia with monosomy 7 is probably a multistep leukemogenic process in which monosomy 7 might be but one of the critical steps. Finally, the prognosis in these cases was poor, suggesting that more aggressive therapy may be needed to improve treatment outcome. Copyright (C) 2000 Elsevier Science Inc.en_HK
dc.languageengen_HK
dc.publisherElsevier Inc. The Journal's web site is located at http://www.elsevier.com/locate/cancergeneen_HK
dc.relation.ispartofCancer Genetics and Cytogeneticsen_HK
dc.rightsCancer Genetics and Cytogenetics. Copyright © Elsevier Inc.en_HK
dc.subject.meshAdolescenten_HK
dc.subject.meshAdulten_HK
dc.subject.meshAge of Onseten_HK
dc.subject.meshChromosomes, Human, Pair 7en_HK
dc.subject.meshFemaleen_HK
dc.subject.meshHumansen_HK
dc.subject.meshIn Situ Hybridization, Fluorescenceen_HK
dc.subject.meshKaryotypingen_HK
dc.subject.meshLeukemia, Myeloid, Acute - genetics - pathologyen_HK
dc.subject.meshMaleen_HK
dc.subject.meshMonosomyen_HK
dc.subject.meshPedigreeen_HK
dc.subject.meshStem Cells - pathologyen_HK
dc.titleFamilial acute myeloid leukemia with monosomy 7: Late onset and involvement of a multipotential progenitor cellen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0165-4608&volume=116&spage=170&epage=173&date=2000&atitle=Familial+acute+myeloid+leukemia+with+monosomy+7:+late+onset+and+involvement+of+a+multipotential+progenitor+cellen_HK
dc.identifier.emailKwong, YL:ylkwong@hku.hken_HK
dc.identifier.authorityKwong, YL=rp00358en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.doi10.1016/S0165-4608(99)00121-1en_HK
dc.identifier.pmid10640152-
dc.identifier.scopuseid_2-s2.0-0033992254en_HK
dc.identifier.hkuros49567en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0033992254&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume116en_HK
dc.identifier.issue2en_HK
dc.identifier.spage170en_HK
dc.identifier.epage173en_HK
dc.identifier.isiWOS:000084354300014-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridKwong, YL=7102818954en_HK
dc.identifier.scopusauthoridNg, MH=35292609300en_HK
dc.identifier.scopusauthoridMa, SK=37020910400en_HK

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