Hemophagocytic lymphohistiocytosis after hematopoietic stem cell transplantation in children: A nationwide survey in Japan

Takeshi Asano, Kazuhiro Kogawa, Akira Morimoto, Yasushi Ishida, Nobuhiro Suzuki, Shouichi Ohga, Kazuko Kudo, Shigeru Ohta, Hiroshi Wakiguchi, Ken Tabuchi, Shunichi Kato, Eiichi Ishii

研究成果: ジャーナルへの寄稿記事

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Background: Hemophagocytic lymphohistiocytosis (HLH) is associated with hypercytokinemia in children. Although HLH can be also observed after hematopoietic stem cell transplantation (HSCT), the incidence and clinical features of HLH after HSCT remain obscure. Procedure: The clinical features of HLH after HSCT (post-HSCT HLH) were investigated in children with malignancies, immune deficiencies, or aplastic anemia. The HLH/Langerhans Cell Histiocytosis (LCH) Committee of the Japanese Society of Pediatric Hematology (JSPH) sent questionnaires to hospitals with JPSH members asking for details of cases in which HLH occurred after HSCT between 1998 and 2008. Results: Among 42 children who were diagnosed with post-HSCT HLH between 1998 and 2008 in Japan, 37 fulfilled our inclusion criteria; of these, 26 were classified as early-onset (onset <30 days after HSCT) and 11 were classified as late-onset (onset >30 days after HSCT). In the early-onset group, the presence of respiratory symptoms, high levels of total bilirubin, and triglycerides at onset and the lack of control of GVHD with tacrolimus were significantly associated with non-resolution of HLH (P<0.05). The survival rate was significantly higher in patients with resolution of HLH than in those without resolution (59% vs. 14%, P<0.05). Conclusions: These findings suggest that early-onset post-HSCT HLH is a specific entity of HLH, and appropriate diagnosis and prompt management need to be established.

元の言語英語
ページ(範囲)110-114
ページ数5
ジャーナルPediatric Blood and Cancer
59
発行部数1
DOI
出版物ステータス出版済み - 7 15 2012
外部発表Yes

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All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Oncology

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