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<p>Systemic Epstein-Barr virus-positive T-cell lymphoma (SEBVTL) of childhood is a rare and aggressive disorder often associated with hemophagocytic lymphohistiocytosis (HLH) and multiorgan failure. We reviewed six pediatric SEBVTL cases treated at Seoul National University Children’s Hospital between 2017 and 2024. All patients presented with HLH and bone marrow involvement. Initial management included the HLH-2004 protocol and chemotherapy. Since December 2022, cooling immunochemotherapy (etoposide and cytarabine) was introduced as a bridge to hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 8.2&#xa0;years, and median follow-up was 8.5&#xa0;months. Three patients treated before 2022 died of progressive disease. Among three patients managed with cooling immunochemotherapy and HSCT, two survived and one died of engraftment failure. The 1-year overall and event-free survival rates were both 33.3%. These findings suggest cooling immunochemotherapy followed by HSCT may improve outcomes in refractory pediatric SEBVTL.</p>

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Cooling immunochemotherapy and hematopoietic stem cell transplantation for refractory systemic epstein-barr virus-positive T-cell lymphoma in children: a single institution experience

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年4月18日

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  • Bo Kyung Kim,
  • Kyung Taek Hong,
  • Jung Yoon Choi,
  • Hyun Jin Park,
  • Hyoung Jin Kang

摘要

Systemic Epstein-Barr virus-positive T-cell lymphoma (SEBVTL) of childhood is a rare and aggressive disorder often associated with hemophagocytic lymphohistiocytosis (HLH) and multiorgan failure. We reviewed six pediatric SEBVTL cases treated at Seoul National University Children’s Hospital between 2017 and 2024. All patients presented with HLH and bone marrow involvement. Initial management included the HLH-2004 protocol and chemotherapy. Since December 2022, cooling immunochemotherapy (etoposide and cytarabine) was introduced as a bridge to hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 8.2 years, and median follow-up was 8.5 months. Three patients treated before 2022 died of progressive disease. Among three patients managed with cooling immunochemotherapy and HSCT, two survived and one died of engraftment failure. The 1-year overall and event-free survival rates were both 33.3%. These findings suggest cooling immunochemotherapy followed by HSCT may improve outcomes in refractory pediatric SEBVTL.