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

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

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type	Academic Article research paper schema:ScholarlyArticle
isDefinedBy	Covid-on-the-Web dataset
title	Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party
Creator	Cho, Bin Kang, Hyoung Koo, Hong Shin, Hee Yoo, Keon Lim, Young Baek, Hee Chung, Nak-Gyun Im, Ho Kim, Hee-Jin Kim, Heung Koh, Kyung-Nam Kook, Hoon Lyu, Chuhl Ryu, Kyung Seo, Jong Yoon, Hoi
source	Medline; PMC
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.
has issue date	2014-07-03(xsd:dateTime)
bibo:doi	10.1111/ejh.12399
bibo:pmid	24935083
has license	no-cc
sha1sum (hex)	17a557b768a7ef6b87bd8c87bbf7ce89d6fb96e9
schema:url	https://doi.org/10.1111/ejh.12399
resource representing a document's title	Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea Histiocytosis Working Party
has PubMed Central identifier	PMC7163615
has PubMed identifier	24935083
schema:publication	Eur J Haematol
resource representing a document's body	covid:17a557b768a7ef6b87bd8c87bbf7ce89d6fb96e9#body_text
is schema:about of	named entity 'study' named entity 'patients' named entity 'mutation' named entity 'transplantation' named entity 'identify' named entity 'Korea' named entity 'pediatric' named entity 'pediatric' named entity 'patients' named entity 'Korea' named entity 'survival' named entity 'disease' named entity 'virus' named entity 'HLH' named entity 'liver dysfunction' named entity 'genetic predisposition' named entity 'HLH' named entity 'HLH' named entity 'ferritin' named entity 'Hemophagocytic lymphohistiocytosis' named entity 'anemia' named entity 'malignancy' named entity '0.01' named entity 'HLH' named entity 'UNC13D' named entity 'immune responses' named entity 'cholestasis' named entity 'HLH' named entity 'genetic mutation' named entity 'HLH' named entity 'UNC13D' named entity 'HLH' named entity 'liver dysfunction' named entity 'Asian' named entity 'hemophagocytosis' named entity 'tropism' named entity 'HLH' named entity 'inflammation' named entity 'hemorrhage' named entity 'UNC13D' named entity 'Griscelli syndrome' named entity '2.52' named entity 'mutation' named entity 'ferritin' named entity 'regression analysis' named entity 'HLH' named entity 'hazard ratio' named entity 'UNC13D' named entity 'HLH' named entity 'bicytopenia' named entity 'HLH' named entity 'antithymocyte globulin' named entity 'PRF1' named entity 'HLH' named entity 'macrophages' named entity 'STXBP2' named entity 'HLH' named entity 'HLH' named entity 'epidemiologic' named entity 'UNC13D' named entity 'HLH' named entity 'infection' named entity 'EBV infection' named entity 'HLH' named entity 'clinical features'

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