Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-02

AUTHORS

Ryu Yanagisawa, Yozo Nakazawa, Kazuyuki Matsuda, Takahiro Yasumi, Hirokazu Kanegane, Shouichi Ohga, Akira Morimoto, Yoshiko Hashii, Masue Imaizumi, Yasuhiro Okamoto, Akiko M. Saito, Keizo Horibe, Eiichi Ishii, HLH/LCH committee members of the Japan Children’s Cancer Group

ABSTRACT

Recent advances in intensive chemo- and immunotherapy have contributed to the outcome of hemophagocytic lymphohistiocytosis (HLH); however, the prognosis of HLH in children differs by HLH subtype. In Japan, secondary HLH, particularly Epstein-Barr virus-associated HLH (EBV-HLH), is the most common HLH subtype. The prognosis of HLH has improved in recent years. We here conducted a prospective study of 73 patients who were treated with HLH-2004 protocol in Japan. EBV-HLH, familial HLH (FHL), and HLH of unknown etiology were seen in 41, 9, and 23 patients, respectively. Patients with resistant or relapsed disease after HLH-2004 treatment and those with FHL received hematopoietic stem cell transplantation (HSCT). The induction rate after initial therapy was 58.9%, and the 3-year overall survival (OS) rate of all patients was 73.9% and differed significantly among those with EBV-HLH, FHL, and HLH of unknown etiology. Of the 17 patients who received HSCT, the 3-year OS rates of those with and without complete resolution before HSCT were 83.3% and 54.5%, respectively. Outcomes in children with HLH who were treated with the same protocol differed among HLH subtypes. Appropriate strategy for each subtype should be established in future studies. More... »

PAGES

206-213

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s12185-018-02572-z

DOI

http://dx.doi.org/10.1007/s12185-018-02572-z

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1110449017

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30535855


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