Song Deli, Wang Jingshi, Zhou Hang, Wu Lin, Zhang Jia, Wang Zhao
Department of Haematology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
Department of Rheumatology, Capital Medical University Affiliated Beijing Friendship Hospital, Beijing, China.
Clin Exp Rheumatol. 2025 Jul;43(7):1294-1302. doi: 10.55563/clinexprheumatol/7l4od4. Epub 2025 Apr 18.
Haemophagocytic lymphohistiocytosis (HLH) with underlying rheumatic diseases (rHLH) is a lethal disease, in which Epstein-Barr virus (EBV) infection is a causative factor. Whether EBV infection is associated with treatment response and prognosis of patients with rHLH remains unclear. This study explored the clinical features of patients with Epstein-Barr virus-positive rHLH.
In this retrospective study, we included 137-patients and divided them into EBV-negative (n=116) and EBV-positive (n=21) groups. We compared the clinical characteristics, treatment responses, and prognoses between the two groups. Propensity score matching (PSM) was used to match patients between groups. Kaplan-Meier analysis was used to elucidate the relationship between the EBV-infected cell type and prognosis.
EBV-positive patients were more likely to have relapsed or refractory rHLH. The survival time of the EBV-negative group was significantly longer than that of the EBV-positive group (p=0.012). Further analysis of EBV-infected lymphocyte subsets revealed a significant decrease in survival in the NK and/or T lymphocyte groups compared to the other cell types (p<0.01).
Patients with EBV-positive rHLH are more likely to experience relapse or refractoriness. For patients with rHLH, prompt testing of EBV-infected lymphocyte subsets should be performed upon EBV infection. An etoposide-based regimen is recommended for patients with EBV-positive rHLH, and rituximab may be effective in patients with refractory or relapsed rHLH with EBV-infected B lymphocytes. However, for patients with EBV-infected NK and/or T lymphocytes, treatment should be aligned with that for EBV-HLH.
伴有潜在风湿性疾病的噬血细胞性淋巴组织细胞增生症（rHLH）是一种致命疾病，其中 Epstein-Barr 病毒（EBV）感染是一个致病因素。EBV 感染是否与 rHLH 患者的治疗反应和预后相关仍不清楚。本研究探讨了 EBV 阳性 rHLH 患者的临床特征。
在这项回顾性研究中，我们纳入了 137 例患者，并将他们分为 EBV 阴性（n = 116）和 EBV 阳性（n = 21）组。我们比较了两组之间的临床特征、治疗反应和预后。采用倾向评分匹配（PSM）在组间匹配患者。采用 Kaplan-Meier 分析阐明 EBV 感染细胞类型与预后之间的关系。
EBV 阳性患者更有可能出现复发或难治性 rHLH。EBV 阴性组的生存时间明显长于 EBV 阳性组（p = 0.012）。对 EBV 感染淋巴细胞亚群的进一步分析显示，与其他细胞类型相比，NK 和/或 T 淋巴细胞组的生存率显著降低（p < 0.01）。
EBV 阳性 rHLH 患者更有可能出现复发或难治情况。对于 rHLH 患者，在 EBV 感染时应及时检测 EBV 感染的淋巴细胞亚群。对于 EBV 阳性 rHLH 患者，推荐采用基于依托泊苷的方案，利妥昔单抗可能对 EBV 感染 B 淋巴细胞的难治性或复发性 rHLH 患者有效。然而，对于 EBV 感染 NK 和/或 T 淋巴细胞的患者，治疗应与 EBV-HLH 的治疗一致。
