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

HLH/LCH committee members of the Japan Children’s Cancer Group

研究成果: ジャーナルへの寄稿記事

1 引用 (Scopus)

抄録

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.

元の言語英語
ページ(範囲)206-213
ページ数8
ジャーナルInternational journal of hematology
109
発行部数2
DOI
出版物ステータス出版済み - 2 20 2019

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Hemophagocytic Lymphohistiocytosis
Japan
Hematopoietic Stem Cell Transplantation
Viruses
Survival Rate
Immunotherapy

All Science Journal Classification (ASJC) codes

  • Hematology

これを引用

Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan. / HLH/LCH committee members of the Japan Children’s Cancer Group.

:: International journal of hematology, 巻 109, 番号 2, 20.02.2019, p. 206-213.

研究成果: ジャーナルへの寄稿記事

HLH/LCH committee members of the Japan Children’s Cancer Group. / Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan. :: International journal of hematology. 2019 ; 巻 109, 番号 2. pp. 206-213.
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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.",
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AU - HLH/LCH committee members of the Japan Children’s Cancer Group

AU - Yanagisawa, Ryu

AU - Nakazawa, Yozo

AU - Matsuda, Kazuyuki

AU - Yasumi, Takahiro

AU - Kanegane, Hirokazu

AU - Ohga, Shouichi

AU - Morimoto, Akira

AU - Hashii, Yoshiko

AU - Ohga, Shoichi

AU - Okamoto, Yasuhiro

AU - Saito, Akiko M.

AU - Horibe, Keizo

AU - Ishii, Eiichi

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