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Haploidentical peripheral blood stem cell transplantation with posttransplant cyclophosphamide for systemic Epstein-Barr virus-positive T-cell lymphoma of childhood

Bone Marrow Transplantation volume 56, pages 1743–1745 (2021)Cite this article

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Epstein-Barr virus-positive T-cell and NK-cell lymphoproliferative diseases (EBV-T/NK-LPD) is categorized into two major groups: systemic Epstein-Barr virus-positive T-cell lymphoma of childhood (SETL) and chronic active EBV infection (CAEBV) [1]. SETL is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype, which develops in children and young adults subsequent to primary acute EBV infection or during the course of CAEBV. Since SETL becomes life-threatening within days to weeks due to hemophagocytosis and multiple organ failure, immediate treatment is often given priority over a confirmed diagnosis [2].

Chemotherapy followed by allogeneic hematopoietic stem cell transplantation (allo-HSCT) is a curative treatment for EBV-T/NK-LPD, regardless of disease category [3]. Due to the rapid progressive nature of the disease, reports of allo-HSCT for SETL are extremely rare: we could find only one case report of a successful HLA-mismatched sibling donor transplantation using myeloablative conditioning (MAC) [4]. Generally, allo-HSCT for SETL is carried out by reference to that for CAEBV.

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Fig. 1: Clinical chart from admission to 2 months posttransplantation.

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Acknowledgements

We thank the medical and nursing staff at the Kobe City Medical Center General Hospital.

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Authors and Affiliations

  1. Department of Hematology, Kobe City Medical Center General Hospital, Kobe, Japan

    Hiroharu Imoto, Satoshi Yoshioka, Nobuhiro Hiramoto, Mari Morita-Fujita & Takayuki Ishikawa

  2. Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Mari Morita-Fujita

  3. Department of Diagnostic Pathology, Kobe City Medical Center General Hospital, Kobe, Japan

    Daisuke Yamashita

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  1. Hiroharu Imoto
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  2. Satoshi Yoshioka
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  3. Nobuhiro Hiramoto
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  4. Mari Morita-Fujita
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  6. Takayuki Ishikawa
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Contributions

HI, SY, NH, and MM-F were the attending physicians. DY contributed to diagnosis as a clinical pathologist. HI and SY wrote the manuscript. TI supervised the case and helped to write the manuscript. All authors revised and approved the manuscript.

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Correspondence to Satoshi Yoshioka.

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The authors declare no competing interests.

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The case was approved by the Clinical Ethics Committee of the Kobe Medical Center General Hospital. The patient signed a written informed consent to publish the case report.

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Imoto, H., Yoshioka, S., Hiramoto, N. et al. Haploidentical peripheral blood stem cell transplantation with posttransplant cyclophosphamide for systemic Epstein-Barr virus-positive T-cell lymphoma of childhood. Bone Marrow Transplant 56, 1743–1745 (2021). https://doi.org/10.1038/s41409-021-01263-4

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