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Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party

DC FieldValueLanguage
dc.contributor.author유철주-
dc.date.accessioned2015-12-28T11:09:04Z-
dc.date.available2015-12-28T11:09:04Z-
dc.date.issued2014-
dc.identifier.issn0902-4441-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/138804-
dc.description.abstractBACKGROUND: We analyzed a nationwide registry of pediatric patients with hemophagocytic lymphohistiocytosis (HLH) in Korea to assess the clinical and genetic features and treatment outcomes in pediatric HLH. METHODS: The Korea Histiocytosis Working Party retrospectively analyzed data on 251 pediatric patients diagnosed with HLH between 1996 and 2011. RESULTS: In the study cohort, 25 cases were categorized with familial HLH, 64 with presumed secondary HLH, and 162 with unspecified HLH. Of 217 evaluable patients, 91 (42%) had concomitant Epstein-Barr virus infection. Of 238 evaluable patients, central nervous system (CNS) involvement, which was more frequent in the familial group, was evident in 81 cases (34%). Genetic tests revealed a predominant UNC13D mutation with a high incidence of two recurrent splicing mutations (c.118-308C>T and c.754-1G>C). The 5-yr overall survival rate was 68% (38% in the familial group and 81% in the presumed secondary group). The 5-yr overall survival rate among 32 patients who underwent allogeneic hematopoietic stem cell transplantation was 64%. In multivariate analysis, a younger age at diagnosis, severe transaminasemia, and a coagulation abnormality were independent prognostic factors for survival. Responses during initial treatments were also significant indicators of outcome. CONCLUSION: Our study showed the unique predominance of a UNC13D mutation and vulnerability to Epstein-Barr virus infection in Korean children with HLH and emphasizes the prognostic significance of age, liver dysfunction, and treatment responses in this disease. A multicenter prospective trial that builds on the present results is warranted to identify subgroups of patients with a poor prognosis and identify optimal treatments.-
dc.description.statementOfResponsibilityopen-
dc.format.extent51~59-
dc.relation.isPartOfEuropean Journal of Haematology-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.titleClinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Pediatrics (소아과학)-
dc.contributor.googleauthorKyung-Nam Koh-
dc.contributor.googleauthorHo Joon Im-
dc.contributor.googleauthorNak-Gyun Chung-
dc.contributor.googleauthorBin Cho-
dc.contributor.googleauthorHyoung Jin Kang-
dc.contributor.googleauthorHee Young Shin-
dc.contributor.googleauthorChuhl Joo Lyu-
dc.contributor.googleauthorKeon Hee Yoo-
dc.contributor.googleauthorHong Hoe Koo-
dc.contributor.googleauthorHee-Jin Kim-
dc.contributor.googleauthorHee Jo Baek-
dc.contributor.googleauthorHoon Kook-
dc.contributor.googleauthorHoi Soo Yoon-
dc.contributor.googleauthorYoung Tak Lim-
dc.contributor.googleauthorHeung Sik Kim-
dc.contributor.googleauthorKyung Ha Ryu-
dc.contributor.googleauthorJong Jin Seo-
dc.contributor.googleauthorKorea Histiocytosis Working Party-
dc.identifier.doi10.1111/ejh.12399-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA02524-
dc.relation.journalcodeJ00822-
dc.identifier.pmid24935083-
dc.identifier.urlhttp://onlinelibrary.wiley.com/doi/10.1111/ejh.12399/abstract-
dc.subject.keywordallogeneic hematopoietic stem cell transplantation-
dc.subject.keywordgenetic mutation-
dc.subject.keywordhemophagocytic lymphohistiocytosis-
dc.subject.keywordprognostic factors-
dc.subject.keywordsurvival-
dc.contributor.alternativeNameLyu, Chuhl Joo-
dc.contributor.affiliatedAuthorLyu, Chuhl Joo-
dc.rights.accessRightsfree-
dc.citation.volume94-
dc.citation.number1-
dc.citation.startPage51-
dc.citation.endPage59-
dc.identifier.bibliographicCitationEuropean Journal of Haematology, Vol.94(1) : 51-59, 2014-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers

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