Pi Yubo, Wang Jingshi, Wang Zhao
Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, 100050, People's Republic of China.
Infect Drug Resist. 2022 Jul 13;15:3751-3756. doi: 10.2147/IDR.S372998. eCollection 2022.
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.
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.
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.
爱泼斯坦-巴尔病毒相关噬血细胞性淋巴组织细胞增生症（EBV-HLH）是一种罕见且侵袭性强的疾病，死亡率高，预后差。迄今为止，异基因造血干细胞移植（allo-HSCT）仍然是治愈EBV-HLH的唯一方法。然而，allo-HSCT后EBV-HLH复发很常见，仍然是一个重大挑战。
一名持续发热一个月的22岁女性因EBV-HLH就诊于我们中心。使用两个周期的L-DEP（聚乙二醇天冬酰胺酶、脂质体阿霉素、依托泊苷和大剂量甲泼尼龙）方案诱导缓解后，该患者接受了人类白细胞抗原（HLA）匹配的同胞allo-HSCT。然而，术后她很快出现疾病复发，所有可能的治疗方案均未取得持续缓解。最后，她接受了信迪利单抗注射，EBV-HLH完全缓解。
我们总结了一例allo-HSCT后复发的EBV-HLH病例，该病例通过程序性细胞死亡蛋白-1（PD-1）抗体成功治疗。需要进一步研究以确定PD-1阻断对allo-HSCT后复发的EBV-HLH是否具有治疗潜力。
