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An analysis of sintilimab combined with ruxolitinib as compassionate therapy for 12 adults with EBV-associated hemophagocytic lymphohistiocytosis

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Abstract

Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a severe hyperinflammatory illness that affects adults and is caused by an EBV infection. Without allogeneic hematopoietic stem cell transplantation (allo-HSCT), the overall survival of adult patients with EBV-HLH is unsatisfactory, necessitating the development of innovative therapeutic approaches. The clinical records of twelve EBV-HLH patients who received sintilimab therapy combined with ruxolitinib on a compassionate basis at the First Affiliated Hospital of Soochow University were retrospectively examined in this investigation. All the patients responded without fever, but three patients relapsed within a week. Among the nine patients achieving complete response (CR), 55.6% (5/9) maintained CR for >4.5 months, and 33.3% (3/9) relapsed following CR. Neither patients with no response (NR) nor relapsed patients were fit for allo-HSCT, and all died soon after discharge. Six patients had clinical CR with a median follow-up of 5 (4.4–14.7) months. There were no documented severe negative effects. Additional information on this innovative treatment for adult EBV-HLH is provided in our report.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

Abbreviations

EBV:

Epstein–Barr virus

HLH:

Hemophagocytic lymphohistiocytosis

HSCT:

Hematopoietic stem cell transplantation

CR:

Complete response

PR:

Partial response

NR:

No response

PD-1:

Programmed death protein-1

RUX:

Ruxolitinib

JAK:

Janus kinase

STAT:

Signal transducer and activator of transcription

CSS:

Cytokine storm syndrome

HL:

Hodgkin lymphoma

MLBCL:

Mediastinal large B-cell lymphoma

GM-CSF:

Granulocyte macrophage colony-stimulating factor

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Acknowledgements

We thank the other doctors on the medical team (Jin Zhou, Yan Wang, Shengwei Lu, Yan Huang) for their work in treating patients.

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Authors and Affiliations

  1. Department of Infectious Diseases, The First Affiliated Hospital of Soochow University, No. 188, Shizi Street, Suzhou, 215006, China

    Ying Xu, Wenting Li, Jianhe Gan & Xiaoping Huang

  2. National Clinical Research Center for Hematologic Diseases, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, No. 188, Shizi Street, Suzhou, 215006, China

    Xuefeng He

Authors
  1. Ying Xu
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  2. Wenting Li
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Contributions

YX contributed to study design and had full access to all data in the study. YX and WL collected patient samples and analyzed the data. YX and XH drafted the manuscript. JG, XH, and XH reviewed the manuscript.

Corresponding authors

Correspondence to Jianhe Gan, Xuefeng He or Xiaoping Huang.

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Ethics approval and consent to participate

Our research was granted an ethics exemption from the ethics committee of the First Affiliated Hospital of Soochow University due to the retrospective and noninterventional nature of the study. The study was based on existing data collected in the course of routine clinical practice, and no additional risks were posed to the patients. Therefore, the need for individual informed consent was waived by the ethics committee.

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Not applicable.

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The authors declare no competing interests.

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Xu, Y., Li, W., Gan, J. et al. An analysis of sintilimab combined with ruxolitinib as compassionate therapy for 12 adults with EBV-associated hemophagocytic lymphohistiocytosis. Ann Hematol 102, 3325–3333 (2023). https://doi.org/10.1007/s00277-023-05476-z

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  • DOI: https://doi.org/10.1007/s00277-023-05476-z

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