Successful Treatment of Relapsed Epstein–Barr Virus-Associated Hemophagocytic Lymphohistiocytosis After Allo-HSCT with PD-1 Blockade: A Case Report

Abstract

Background

Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a rare and aggressive disease with high mortality and poor prognosis. To date, allogeneic hematopoietic stem cell transplantation (allo-HSCT) remains the only way to cure EBV-HLH. However, relapse of EBV-HLH after allo-HSCT is common and remains a major challenge.

Case Presentation

A 22-year-old woman with persistent fever for a month presented to our center with EBV-HLH. After induction of remission using two cycles of the L-DEP (PEG-aspargase, liposomal doxorubicin, etoposide, and high-dose methylprednisolone) regimen, the patient underwent an human leukocyte antigen (HLA)-identical sibling allo-HSCT. However, she experienced disease relapse soon after the procedure, and none of the possible treatment options achieved a sustained response. Finally, she received a sintilimab injection and achieved complete resolution of EBV-HLH.

Conclusion

We summarize a case of relapsed EBV-HLH after allo-HSCT that was successfully treated with a programmed cell death protein-1 (PD-1) antibody. Further studies are needed to determine whether PD-1 blockade has therapeutic potential for relapsed EBV-HLH after allo-HSCT.

Keywords:

• Epstein–Barr virus
• allogeneic hematopoietic stem cell transplantation
• PD-1 antibody
• graft-versus-host disease

Ethics Approval and Informed Consent

This report was approved by the ethics committee of Beijing Friendship Hospital.

The patient provided written informed consent and agreed to the publication of the case.

Acknowledgments

We thank all authors for their contributions to this work.

Author Contributions

All authors made substantial contributions to conception and design, acquisition of data, or analysis and interpretation of data; took part in drafting the article or revising it critically for important intellectual content; agreed to submit to the current journal; gave final approval of the version to be published; and agreed to be accountable for all aspects of the work.

Disclosure

The authors declare no conflicts of interest in this work.

Additional information

Funding

There is no funding to report.