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Article: Transient myeloproliferative disorder in a Down's neonate with rearranged T-cell receptor β gene and evidence of in vivo maturation demonstrated by dual-colour flow cytometric DNA ploidy analysis

TitleTransient myeloproliferative disorder in a Down's neonate with rearranged T-cell receptor β gene and evidence of in vivo maturation demonstrated by dual-colour flow cytometric DNA ploidy analysis
Authors
Issue Date1993
PublisherNature Publishing Group. The Journal's web site is located at http://www.nature.com/leu
Citation
Leukemia, 1993, v. 7 n. 10, p. 1667-1671 How to Cite?
AbstractNeonates with Down's syndrome may develop a transient myeloproliferative disorder (TMPD) which on presentation is indistinguishable from acute leukemia, with the difference manifest only on follow-up. The clinical course is one of spontaneous remission in TMPD and relentless progression in leukemia. We describe a Down's neonate presenting with hyperleucocytosis and circulating blasts which were positive for CD34, myeloid (CD33), megakaryocytic (CD41,CD42b,CD61), and T-lineage (CD3,CD7), but not B-lineage, associated antigens. Clonal rearrangement of the T-cell receptor β (TCR(β)) gene was found, with the immunoglobulin heavy chain gene in germline configuration, showing the disease to be a clonal proliferation of a multipotential stem cell involving the myeloid and T lineages. Dual-colour flow cytometric DNA ploidy analysis of CD41 positive blasts showed initially a predominant 2N population, but polyploidization to 6N and 8N cells was found on follow-up, concomitant with a progressive decrease in circulating blasts, suggesting maturation of the abnormal clone and a provisional diagnosis of TMPD. This was shown by the eventual resumption of normal haemopoiesis with the disappearance of blasts and the clonally rearranged TCR(β) gene.
Persistent Identifierhttp://hdl.handle.net/10722/147974
ISSN
2015 Impact Factor: 12.104
2015 SCImago Journal Rankings: 5.142
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorKwong, YLen_HK
dc.contributor.authorCheng, Gen_HK
dc.contributor.authorTang, TSen_HK
dc.contributor.authorRobertson, EPen_HK
dc.contributor.authorLee, CPen_HK
dc.contributor.authorChan, LCen_HK
dc.date.accessioned2012-05-29T06:10:11Z-
dc.date.available2012-05-29T06:10:11Z-
dc.date.issued1993en_HK
dc.identifier.citationLeukemia, 1993, v. 7 n. 10, p. 1667-1671en_HK
dc.identifier.issn0887-6924en_HK
dc.identifier.urihttp://hdl.handle.net/10722/147974-
dc.description.abstractNeonates with Down's syndrome may develop a transient myeloproliferative disorder (TMPD) which on presentation is indistinguishable from acute leukemia, with the difference manifest only on follow-up. The clinical course is one of spontaneous remission in TMPD and relentless progression in leukemia. We describe a Down's neonate presenting with hyperleucocytosis and circulating blasts which were positive for CD34, myeloid (CD33), megakaryocytic (CD41,CD42b,CD61), and T-lineage (CD3,CD7), but not B-lineage, associated antigens. Clonal rearrangement of the T-cell receptor β (TCR(β)) gene was found, with the immunoglobulin heavy chain gene in germline configuration, showing the disease to be a clonal proliferation of a multipotential stem cell involving the myeloid and T lineages. Dual-colour flow cytometric DNA ploidy analysis of CD41 positive blasts showed initially a predominant 2N population, but polyploidization to 6N and 8N cells was found on follow-up, concomitant with a progressive decrease in circulating blasts, suggesting maturation of the abnormal clone and a provisional diagnosis of TMPD. This was shown by the eventual resumption of normal haemopoiesis with the disappearance of blasts and the clonally rearranged TCR(β) gene.en_HK
dc.languageengen_US
dc.publisherNature Publishing Group. The Journal's web site is located at http://www.nature.com/leuen_HK
dc.relation.ispartofLeukemiaen_HK
dc.subject.meshBone Marrow - Chemistry - Physiologyen_US
dc.subject.meshDna - Geneticsen_US
dc.subject.meshDown Syndrome - Blood - Complications - Geneticsen_US
dc.subject.meshFlow Cytometry - Methodsen_US
dc.subject.meshGene Rearrangement - Geneticsen_US
dc.subject.meshGene Rearrangement, Beta-Chain T-Cell Antigen Receptor - Geneticsen_US
dc.subject.meshGenes, Immunoglobulin - Geneticsen_US
dc.subject.meshHumansen_US
dc.subject.meshImmunophenotypingen_US
dc.subject.meshInfant, Newbornen_US
dc.subject.meshKaryotypingen_US
dc.subject.meshLeukocytosis - Blooden_US
dc.subject.meshMaleen_US
dc.subject.meshMyeloproliferative Disorders - Complications - Diagnosis - Geneticsen_US
dc.subject.meshPloidiesen_US
dc.titleTransient myeloproliferative disorder in a Down's neonate with rearranged T-cell receptor β gene and evidence of in vivo maturation demonstrated by dual-colour flow cytometric DNA ploidy analysisen_HK
dc.typeArticleen_HK
dc.identifier.emailKwong, YL:ylkwong@hku.hken_HK
dc.identifier.emailChan, LC:chanlc@hkucc.hku.hken_HK
dc.identifier.authorityKwong, YL=rp00358en_HK
dc.identifier.authorityChan, LC=rp00373en_HK
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.pmid8412331-
dc.identifier.scopuseid_2-s2.0-0027421833en_HK
dc.identifier.hkuros30272-
dc.identifier.volume7en_HK
dc.identifier.issue10en_HK
dc.identifier.spage1667en_HK
dc.identifier.epage1671en_HK
dc.identifier.isiWOS:A1993MD20600030-
dc.publisher.placeUnited Kingdomen_HK
dc.identifier.scopusauthoridKwong, YL=7102818954en_HK
dc.identifier.scopusauthoridCheng, G=35313354500en_HK
dc.identifier.scopusauthoridTang, TS=7401988840en_HK
dc.identifier.scopusauthoridRobertson, EP=7202391529en_HK
dc.identifier.scopusauthoridLee, CP=7410149538en_HK
dc.identifier.scopusauthoridChan, LC=7403540707en_HK

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