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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
2012 Impact Factor: 0.068
2012 SCImago Journal Rankings: 0.107
 
ReferencesReferences in Scopus
 
DC FieldValue
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
2012 Impact Factor: 0.068
2012 SCImago Journal Rankings: 0.107
 
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
 
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<contributor.author>Cheuk, DKL</contributor.author>
<contributor.author>Li, CH</contributor.author>
<contributor.author>So, CC</contributor.author>
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Author Affiliations
  1. The University of Hong Kong Li Ka Shing Faculty of Medicine
  2. Tuen Mun Hospital