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    Please use this identifier to cite or link to this item: http://ir.nhri.org.tw/handle/3990099045/4085


    Title: Comprehensive analyses and characterization of haemophagocytic lymphohistiocytosis in Vietnamese children
    Authors: My, LT;Lien, LB;Hsieh, WC;Imamura, T;Anh, TNK;Anh, PNL;Hung, NT;Tseng, FC;Chi, CY;Dao, NTH;Le, DTM;Thinh, LQ;Tung, TT;Imashuku, S;Thuong, TC;Su, IJ
    Contributors: Division of Infectious Diseases
    Abstract: Haemophagocytic lymphohistiocytosis (HLH) is a fatal haematological disorder with diverse aetiology. This prospective study was undertaken to characterize HLH cases in Vietnamese children. Clinical and laboratory data, genetic analyses and outcome of the HLH patients were analysed. A total of 33 patients were enrolled from March 2007 to December 2008, with a median age of 3 years. Mutations of the SH2D1A (SAP) and PRF1 genes were detected in one patient, respectively. The virus association was high, up to 63·6% (21/33), including Epstein-Barr virus (19/33), cytomegalovirus (2/33) and dengue virus (2/33). Five patients had malignant lymphoma and two had autoimmune diseases. Twenty-eight patients were treated according to the HLH-2004 protocol. The first response rate was 64·3% (18/28), with an early death rate of 35·7% (10/28). High levels of interferon-γ, interleukin-10, MIG and interferon-inducible protein-10 (IP-10) were associated with early mortality (P < 0·05). Reactivation among the responders was high (9/18) and the uneventful resolution was low (3/18) after a median follow-up of 35 weeks. In conclusion, the majority of HLH cases are associated with virus infections in Vietnamese children. Familial HLH is rare. The frequent reactivation and high mortality demands a more appropriate therapeutic regimen in tropical areas like Vietnam.
    Date: 2010-01
    Relation: British Journal of Haematology. 2010 Jan;148(2):301-310.
    Link to: http://dx.doi.org/10.1111/j.1365-2141.2009.07957.x
    JIF/Ranking 2023: http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcAuth=NHRI&SrcApp=NHRI_IR&KeyISSN=0007-1048&DestApp=IC2JCR
    Cited Times(WOS): https://www.webofscience.com/wos/woscc/full-record/WOS:000272884100013
    Cited Times(Scopus): http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=72649096727
    Appears in Collections:[蘇益仁(2002-2015)] 期刊論文
    [齊嘉鈺] 期刊論文
    [曾凡真] 期刊論文

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