Cited 55 times in
Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party
DC Field | Value | Language |
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dc.contributor.author | 유철주 | - |
dc.date.accessioned | 2015-12-28T11:09:04Z | - |
dc.date.available | 2015-12-28T11:09:04Z | - |
dc.date.issued | 2015 | - |
dc.identifier.issn | 0902-4441 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/138804 | - |
dc.description.abstract | BACKGROUND: 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.statementOfResponsibility | open | - |
dc.relation.isPartOf | EUROPEAN JOURNAL OF HAEMATOLOGY | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.title | Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine (의과대학) | - |
dc.contributor.department | Dept. of Pediatrics (소아과학) | - |
dc.contributor.googleauthor | Kyung-Nam Koh | - |
dc.contributor.googleauthor | Ho Joon Im | - |
dc.contributor.googleauthor | Nak-Gyun Chung | - |
dc.contributor.googleauthor | Bin Cho | - |
dc.contributor.googleauthor | Hyoung Jin Kang | - |
dc.contributor.googleauthor | Hee Young Shin | - |
dc.contributor.googleauthor | Chuhl Joo Lyu | - |
dc.contributor.googleauthor | Keon Hee Yoo | - |
dc.contributor.googleauthor | Hong Hoe Koo | - |
dc.contributor.googleauthor | Hee-Jin Kim | - |
dc.contributor.googleauthor | Hee Jo Baek | - |
dc.contributor.googleauthor | Hoon Kook | - |
dc.contributor.googleauthor | Hoi Soo Yoon | - |
dc.contributor.googleauthor | Young Tak Lim | - |
dc.contributor.googleauthor | Heung Sik Kim | - |
dc.contributor.googleauthor | Kyung Ha Ryu | - |
dc.contributor.googleauthor | Jong Jin Seo | - |
dc.contributor.googleauthor | Korea Histiocytosis Working Party | - |
dc.identifier.doi | 10.1111/ejh.12399 | - |
dc.admin.author | false | - |
dc.admin.mapping | false | - |
dc.contributor.localId | A02524 | - |
dc.relation.journalcode | J00822 | - |
dc.identifier.eissn | 1600-0609 | - |
dc.identifier.pmid | 24935083 | - |
dc.identifier.url | http://onlinelibrary.wiley.com/doi/10.1111/ejh.12399/abstract | - |
dc.subject.keyword | allogeneic hematopoietic stem cell transplantation | - |
dc.subject.keyword | genetic mutation | - |
dc.subject.keyword | hemophagocytic lymphohistiocytosis | - |
dc.subject.keyword | prognostic factors | - |
dc.subject.keyword | survival | - |
dc.contributor.alternativeName | Lyu, Chuhl Joo | - |
dc.contributor.affiliatedAuthor | Lyu, Chuhl Joo | - |
dc.rights.accessRights | free | - |
dc.citation.volume | 94 | - |
dc.citation.number | 1 | - |
dc.citation.startPage | 51 | - |
dc.citation.endPage | 59 | - |
dc.identifier.bibliographicCitation | EUROPEAN JOURNAL OF HAEMATOLOGY, Vol.94(1) : 51-59, 2015 | - |
dc.identifier.rimsid | 53803 | - |
dc.type.rims | ART | - |
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