Haploidentical hematopoietic stem cell transplantation for adult patients with Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis

Abstract

To assess the efficacy of haploidentical hematopoietic stem cell transplantation (HSCT) in adult patients with Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), we retrospectively analyzed 30 cases that presented at our institution. At the time of HSCT, 20 patients (66.7%) had achieved a response after receiving HLH-94 or salvage therapies. All patients underwent myeloablative conditioning followed by peripheral blood HSCT from their related, haploidentical donors. Twenty-six patients (86.7%) achieved donor cell engraftment. Of these, 23 (88.5%) achieved complete chimerism and three (11.5%) demonstrated mixed chimerism. Reactivated EBV infection was found in 25 (96.2%). Acute graft-versus-host disease occurred in 18 (69.2%), with grade I–II in 11 patients and grade III–IV in seven. Chronic graft-versus-host disease occurred in six (23.1%). Nineteen patients survived until the end of follow-up. The three-year overall survival rate was 63.3%. Our results indicate that haploidentical HSCT is an effective treatment for adult patients with EBV-HLH.

Keywords:

• Hemophagocytic lymphohistiocytosis
• adult
• Epstein–Barr virus
• haploidentical
• allogeneic hematopoietic stem cell transplantation

Acknowledgements

The authors are grateful to the all colleagues in the Department of Hematology of Beijing Friendship Hospital for their excellent assistance.

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article online at https://doi/org/10.1080/10428194.2017.1330467.

Additional information

Funding

This work was supported by the National Natural Science Foundation of Youth Project of China [grant number 81401627]; the Capital Health Research and Medical Development Foundation [grant number 2014-4-2025]; Beijing Natural Science Foundation [grant number 7132087].