Article: Challenges in the management of juvenile myelomonocytic leukaemia in Hong Kong over the past two decades

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TitleChallenges in the management of juvenile myelomonocytic leukaemia in Hong Kong over the past two decades
AuthorsSurapolchai, P1
Ha, SY1
Chiang, AKS1
Cheuk, DKL1
Li, CH2
So, CC1
Wan, TSK1
Chan, GCF1
KeywordsCase Series
Diagnosis
Juvenile Myelomonocytic Leukaemia
Treatment Outcome
Issue Date2012
PublisherMedcom Limited. The Journal's web site is located at http://www.hkjpaed.org/index.asp
CitationHong Kong Journal Of Paediatrics, 2012, v. 17 n. 3, p. 148-155 [How to Cite?]
AbstractJuvenile myelomonocytic leukaemia (JMML) is a rare myeloid malignancy of childhood. The diagnosis and treatment of this disease still remain as major clinical challenges. We aim to describe the clinical and pathological findings, as well as treatment outcomes of eight patients with JMML who received treatment in two hospitals in Hong Kong between 1993 and 2011. One patient with Noonan syndrome showed spontaneous resolution of disease. Four patients underwent allogeneic haematopoietic stem cell transplantation during 1996 to 2007. Three of them died of post-transplant relapse and refractory disease or transplant-related toxicity. The surviving transplanted patient had chronic graft-versus-host disease for 4 years, and eventually showed evidence of disease relapse with documented mixed chimerism. The other three patients who received supportive treatment were alive with persistent disease. Our future works should focus on optimising therapy and improving treatment outcome for JMML patients.
DescriptionOpen Access Journal
ISSN1013-9923
2011 Impact Factor: 0.027
2011 SCImago Journal Rankings: 0.029
ReferencesReferences in Scopus
DC Field
Value
dc.contributor.authorSurapolchai, P
dc.contributor.authorHa, SY
dc.contributor.authorChiang, AKS
dc.contributor.authorCheuk, DKL
dc.contributor.authorLi, CH
dc.contributor.authorSo, CC
dc.contributor.authorWan, TSK
dc.contributor.authorChan, GCF
dc.date.accessioned2012-10-30T06:09:14Z
dc.date.available2012-10-30T06:09:14Z
dc.date.issued2012
dc.description.abstractJuvenile myelomonocytic leukaemia (JMML) is a rare myeloid malignancy of childhood. The diagnosis and treatment of this disease still remain as major clinical challenges. We aim to describe the clinical and pathological findings, as well as treatment outcomes of eight patients with JMML who received treatment in two hospitals in Hong Kong between 1993 and 2011. One patient with Noonan syndrome showed spontaneous resolution of disease. Four patients underwent allogeneic haematopoietic stem cell transplantation during 1996 to 2007. Three of them died of post-transplant relapse and refractory disease or transplant-related toxicity. The surviving transplanted patient had chronic graft-versus-host disease for 4 years, and eventually showed evidence of disease relapse with documented mixed chimerism. The other three patients who received supportive treatment were alive with persistent disease. Our future works should focus on optimising therapy and improving treatment outcome for JMML patients.
dc.description.naturelink_to_OA_fulltext
dc.descriptionOpen Access Journal
dc.identifier.citationHong Kong Journal Of Paediatrics, 2012, v. 17 n. 3, p. 148-155 [How to Cite?]
dc.identifier.epage155
dc.identifier.hkuros209851
dc.identifier.issn1013-9923
2011 Impact Factor: 0.027
2011 SCImago Journal Rankings: 0.029
dc.identifier.issue3
dc.identifier.scopuseid_2-s2.0-84864392566
dc.identifier.spage148
dc.identifier.urihttp://hdl.handle.net/10722/170470
dc.identifier.volume17
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.subjectCase Series
dc.subjectDiagnosis
dc.subjectJuvenile Myelomonocytic Leukaemia
dc.subjectTreatment Outcome
dc.titleChallenges in the management of juvenile myelomonocytic leukaemia in Hong Kong over the past two decades
dc.typeArticle
Author Affiliations
  1. The University of Hong Kong Li Ka Shing Faculty of Medicine
  2. Tuen Mun Hospital