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Article: A small cohort review of neonatal transient myeloproliferative disease in Chinese children
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TitleA small cohort review of neonatal transient myeloproliferative disease in Chinese children
 
AuthorsXiong, H2
Ha, SY2
Chiang, AKS1
Cheuk, DKL1
Zeng, LK2
Chan, GCF1
 
KeywordsAcute megakaryoblastic leukaemia (AMKL)
Down syndrome
Transient myeloproliferative disease
Trisomy 21
 
Issue Date2011
 
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
 
CitationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 4, p. 258-263 [How to Cite?]
 
AbstractBackground: Neonates with constitutional trisomy 21 are predisposed to develop transient myeloproliferative disease (TMD). TMD is characterised by a rapid accumulation of blast cells during the first few days of life followed by spontaneous resolution. Around 20% to 30% of them subsequently evolve into acute megakaryoblastic leukaemia (AMKL or FAB M7). Objective: To examine the natural history and biological characteristics of neonatal TMD, the clinical characteristic associated with subsequent AMKL, and the prognosis of AMKL with constitutional trisomy 21 in Chinese children. Methods: We retrospectively reviewed the charts of 4 neonates with trisomy 21 and TMD and compared them with that of the literature. Results: Trisomy 21 was the only cytogenetic abnormality identified in the blast cells of the 4 patients. In all of the neonates, peripheral blast cells cleared spontaneously, blood counts normalised and complete remission ensued without chemotherapy. Three of the 4 neonates developed AMKL at a mean age of 15 months of age and they were treated with chemotherapy. All achieved and maintained complete remission for a mean duration of 8 years (range 6.1-10.4 years). The remaining patient was found to have trisomy 21 only in the blast cells and he has normal phenotype without any Down's stigmata. Conclusion: Neonatal TMD is a unique clinical syndrome associated with spontaneous remission but with a high chance of developing AMKL subsequently. Interestingly, such AMKL are chemosensitive and can achieve long term remission with chemotherapy alone. Further research should focus on the role of genetic interactions of trisomy 21 in leukaemogenesis and on identifying specific therapeutic targets. Multicentre collaborative study has been conducting and risk stratification approach has been applied to minimise the therapy related toxicity currently.
 
DescriptionThe article can be viewed at http://www.hkjpaed.org/pdf/2011;16;258-263.pdf
 
ISSN1013-9923
2013 Impact Factor: 0.106
2013 SCImago Journal Rankings: 0.126
 
ReferencesReferences in Scopus
 
DC FieldValue
dc.contributor.authorXiong, H
 
dc.contributor.authorHa, SY
 
dc.contributor.authorChiang, AKS
 
dc.contributor.authorCheuk, DKL
 
dc.contributor.authorZeng, LK
 
dc.contributor.authorChan, GCF
 
dc.date.accessioned2011-11-24T10:04:30Z
 
dc.date.available2011-11-24T10:04:30Z
 
dc.date.issued2011
 
dc.description.abstractBackground: Neonates with constitutional trisomy 21 are predisposed to develop transient myeloproliferative disease (TMD). TMD is characterised by a rapid accumulation of blast cells during the first few days of life followed by spontaneous resolution. Around 20% to 30% of them subsequently evolve into acute megakaryoblastic leukaemia (AMKL or FAB M7). Objective: To examine the natural history and biological characteristics of neonatal TMD, the clinical characteristic associated with subsequent AMKL, and the prognosis of AMKL with constitutional trisomy 21 in Chinese children. Methods: We retrospectively reviewed the charts of 4 neonates with trisomy 21 and TMD and compared them with that of the literature. Results: Trisomy 21 was the only cytogenetic abnormality identified in the blast cells of the 4 patients. In all of the neonates, peripheral blast cells cleared spontaneously, blood counts normalised and complete remission ensued without chemotherapy. Three of the 4 neonates developed AMKL at a mean age of 15 months of age and they were treated with chemotherapy. All achieved and maintained complete remission for a mean duration of 8 years (range 6.1-10.4 years). The remaining patient was found to have trisomy 21 only in the blast cells and he has normal phenotype without any Down's stigmata. Conclusion: Neonatal TMD is a unique clinical syndrome associated with spontaneous remission but with a high chance of developing AMKL subsequently. Interestingly, such AMKL are chemosensitive and can achieve long term remission with chemotherapy alone. Further research should focus on the role of genetic interactions of trisomy 21 in leukaemogenesis and on identifying specific therapeutic targets. Multicentre collaborative study has been conducting and risk stratification approach has been applied to minimise the therapy related toxicity currently.
 
dc.description.naturelink_to_subscribed_fulltext
 
dc.descriptionThe article can be viewed at http://www.hkjpaed.org/pdf/2011;16;258-263.pdf
 
dc.identifier.citationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 4, p. 258-263 [How to Cite?]
 
dc.identifier.epage263
 
dc.identifier.hkuros197753
 
dc.identifier.hkuros198987
 
dc.identifier.issn1013-9923
2013 Impact Factor: 0.106
2013 SCImago Journal Rankings: 0.126
 
dc.identifier.issue4
 
dc.identifier.scopuseid_2-s2.0-80054808325
 
dc.identifier.spage258
 
dc.identifier.urihttp://hdl.handle.net/10722/143377
 
dc.identifier.volume16
 
dc.languageeng
 
dc.publisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
 
dc.publisher.placeHong Kong
 
dc.relation.ispartofHong Kong Journal of Paediatrics
 
dc.relation.referencesReferences in Scopus
 
dc.subjectAcute megakaryoblastic leukaemia (AMKL)
 
dc.subjectDown syndrome
 
dc.subjectTransient myeloproliferative disease
 
dc.subjectTrisomy 21
 
dc.titleA small cohort review of neonatal transient myeloproliferative disease in Chinese children
 
dc.typeArticle
 
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<item><contributor.author>Xiong, H</contributor.author>
<contributor.author>Ha, SY</contributor.author>
<contributor.author>Chiang, AKS</contributor.author>
<contributor.author>Cheuk, DKL</contributor.author>
<contributor.author>Zeng, LK</contributor.author>
<contributor.author>Chan, GCF</contributor.author>
<date.accessioned>2011-11-24T10:04:30Z</date.accessioned>
<date.available>2011-11-24T10:04:30Z</date.available>
<date.issued>2011</date.issued>
<identifier.citation>Hong Kong Journal Of Paediatrics, 2011, v. 16 n. 4, p. 258-263</identifier.citation>
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<description.abstract>Background: Neonates with constitutional trisomy 21 are predisposed to develop transient myeloproliferative disease (TMD). TMD is characterised by a rapid accumulation of blast cells during the first few days of life followed by spontaneous resolution. Around 20% to 30% of them subsequently evolve into acute megakaryoblastic leukaemia (AMKL or FAB M7). Objective: To examine the natural history and biological characteristics of neonatal TMD, the clinical characteristic associated with subsequent AMKL, and the prognosis of AMKL with constitutional trisomy 21 in Chinese children. Methods: We retrospectively reviewed the charts of 4 neonates with trisomy 21 and TMD and compared them with that of the literature. Results: Trisomy 21 was the only cytogenetic abnormality identified in the blast cells of the 4 patients. In all of the neonates, peripheral blast cells cleared spontaneously, blood counts normalised and complete remission ensued without chemotherapy. Three of the 4 neonates developed AMKL at a mean age of 15 months of age and they were treated with chemotherapy. All achieved and maintained complete remission for a mean duration of 8 years (range 6.1-10.4 years). The remaining patient was found to have trisomy 21 only in the blast cells and he has normal phenotype without any Down&apos;s stigmata. Conclusion: Neonatal TMD is a unique clinical syndrome associated with spontaneous remission but with a high chance of developing AMKL subsequently. Interestingly, such AMKL are chemosensitive and can achieve long term remission with chemotherapy alone. Further research should focus on the role of genetic interactions of trisomy 21 in leukaemogenesis and on identifying specific therapeutic targets. Multicentre collaborative study has been conducting and risk stratification approach has been applied to minimise the therapy related toxicity currently.</description.abstract>
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Author Affiliations
  1. The University of Hong Kong Li Ka Shing Faculty of Medicine
  2. Wuhan Children's Hospital