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Article: A small cohort review of neonatal transient myeloproliferative disease in Chinese children

TitleA small cohort review of neonatal transient myeloproliferative disease in Chinese children
Authors
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
Citation
Hong 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.
Persistent Identifierhttp://hdl.handle.net/10722/143377
ISSN
2014 Impact Factor: 0.133
2013 SCImago Journal Rankings: 0.126
References

 

DC FieldValueLanguage
dc.contributor.authorXiong, Hen_HK
dc.contributor.authorHa, SYen_HK
dc.contributor.authorChiang, AKSen_HK
dc.contributor.authorCheuk, DKLen_HK
dc.contributor.authorZeng, LKen_HK
dc.contributor.authorChan, GCFen_HK
dc.date.accessioned2011-11-24T10:04:30Z-
dc.date.available2011-11-24T10:04:30Z-
dc.date.issued2011en_HK
dc.identifier.citationHong Kong Journal Of Paediatrics, 2011, v. 16 n. 4, p. 258-263en_HK
dc.identifier.issn1013-9923en_HK
dc.identifier.urihttp://hdl.handle.net/10722/143377-
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.en_HK
dc.languageengen_US
dc.publisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.aspen_HK
dc.relation.ispartofHong Kong Journal of Paediatricsen_HK
dc.subjectAcute megakaryoblastic leukaemia (AMKL)en_HK
dc.subjectDown syndromeen_HK
dc.subjectTransient myeloproliferative diseaseen_HK
dc.subjectTrisomy 21en_HK
dc.titleA small cohort review of neonatal transient myeloproliferative disease in Chinese childrenen_HK
dc.typeArticleen_HK
dc.identifier.emailChiang, AKS:chiangak@hkucc.hku.hken_HK
dc.identifier.emailChan, GCF:gcfchan@hkucc.hku.hken_HK
dc.identifier.authorityChiang, AKS=rp00403en_HK
dc.identifier.authorityChan, GCF=rp00431en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.scopuseid_2-s2.0-80054808325en_HK
dc.identifier.hkuros197753en_US
dc.identifier.hkuros198987-
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-80054808325&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume16en_HK
dc.identifier.issue4en_HK
dc.identifier.spage258en_HK
dc.identifier.epage263en_HK
dc.publisher.placeHong Kongen_HK
dc.identifier.scopusauthoridXiong, H=36438003100en_HK
dc.identifier.scopusauthoridHa, SY=7202501115en_HK
dc.identifier.scopusauthoridChiang, AKS=7101623534en_HK
dc.identifier.scopusauthoridCheuk, DKL=8705936100en_HK
dc.identifier.scopusauthoridZeng, LK=54387646400en_HK
dc.identifier.scopusauthoridChan, GCF=16160154400en_HK

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