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Chronic active Epstein-Barr virus infection involving gastrointestinal tract with hemophagocytic lymphohistiocytosis

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Abstract

Chronic active EBV infection (CAEBV) is a lymphoproliferative disorder of T- or NK-cell type in Asian countries. CAEBV involving the gastrointestinal tract (GI CAEBV) is a rare condition with poor prognosis that may rapidly progress with hemophagocytic lymphohistiocytosis (HLH) and life-threatening complications such as GI bleeding and/or perforation. The approach to CAEBV with GI tract involvement (GI CAEBV) is still an unmet clinical need. In this case series study, we summarized the clinical features, treatment, and prognosis of seven cases of GI CAEBV with HLH, particularly focusing on its prognosis and the possible salvage therapy combining surgery, novel therapeutic agents, and/or autologous(auto-) hematopoietic stem cell transplantation (HSCT) based on successful cases from our center. GI CAEBV is often misdiagnosed as inflammatory bowel diseases and certain infections. The key to its early recognition is the integrative consideration of its systemic manifestation, serum virology, endoscopic, and imaging findings along with pathology. Surgical intervention should not be hesitated when life-threatening GI complications occur. Resection of the involved bowel segment is an effective way of controlling bleeding and reducing tumor burden. In addition to upfront allogeneic HSCT, new therapeutic modalities including PD-1 antibody and auto-HSCT may be effective in certain patients.

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Data availability

All revelant summary data generated during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

This work is funded by the Natural Science Foundation of Sichuan Province (2022NSFSC1463).

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Authors and Affiliations

  1. Department of Hematology, West China Hospital of Sichuan University, Chengdu, 610041, China

    Kai Shen, Xiao Shuai, Jianjun Li, Jiazhuo Liu, Ting Liu, Ting Niu & Hongbing Ma

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  1. Kai Shen
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  2. Xiao Shuai
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  3. Jianjun Li
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  4. Jiazhuo Liu
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  5. Ting Liu
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  6. Ting Niu
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  7. Hongbing Ma
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Corresponding author

Correspondence to Hongbing Ma.

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Ethics approval

This study was conducted with the approval of the ethics committee of The West China Hospital of Sichuan University. All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Not needed due to the retrospective nature of this study.

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The authors declare no competing interests.

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Shen, K., Shuai, X., Li, J. et al. Chronic active Epstein-Barr virus infection involving gastrointestinal tract with hemophagocytic lymphohistiocytosis. Ann Hematol 102, 45–53 (2023). https://doi.org/10.1007/s00277-022-05081-6

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  • DOI: https://doi.org/10.1007/s00277-022-05081-6

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