Purpose

T cell-Epstein-Barr virus–associated hemophagocytic lymphohistiocytosis (T cell-EBV-HLH) is prevalent in East Asia and has poor prognosis. Understanding of this disease is limited, and literature regarding prevalence in North America is scarce. Herein, we summarize our experience.

Methods

A retrospective analysis of T cell-EBV-HLH patients admitted to Children’s Healthcare of Atlanta (GA, USA) from 2010 to 2020 was conducted. Additional immune studies were completed in a subset of patients.

Results

We report 15 patients (10 months–19 years of age) diagnosed with T cell-EBV-HLH. Nine patients were Hispanic, and the majority did not have primary HLH (p-HLH) gene defects. Soluble interleukin-2 receptor levels in T cell-EBV-HLH were significantly higher than other forms of secondary-HLH but comparable to p-HLH, and it correlated with disease severity at presentation. Natural killer cell function was decreased in most patients despite a negative workup for p-HLH. Depending on disease severity, initial therapy included dexamethasone or dexamethasone and etoposide. Refractory patients were managed with blended regimens that included one or more of the following therapies: combination chemotherapy, alemtuzumab, emapalumab, and nivolumab. Rituximab did not appreciably decrease EBV viremia in most patients. Non-critically ill patients responded well to immunosuppressive therapy and are long-term survivors without undergoing allogeneic hematopoietic stem cell transplantation (HSCT). Alemtuzumab resulted in inflammation flare in two of the three patients. Three patients underwent allogeneic HSCT, with disease relapse noted in one. At a median follow-up of 3 years, 10 of the 15 patients are alive.

Conclusion

T cell-EBV-HLH occurs in the USA among the non-Asian populations, especially in those who are Hispanic.

中文翻译：

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T 细胞-爱泼斯坦-巴尔病毒相关的噬血细胞性淋巴组织细胞增多症 (HLH) 发生在非亚洲人中，并且与与原发性 HLH 相当的 T 细胞激活状态有关

目的

T 细胞-爱泼斯坦-巴尔病毒相关的噬血细胞性淋巴组织细胞增多症（T 细胞-EBV-HLH）在东亚流行，预后较差。对这种疾病的了解有限，关于北美流行的文献也很少。在此，我们总结一下我们的经验。

方法

对 2010 年至 2020 年亚特兰大儿童保健中心（美国乔治亚州）收治的 T 细胞-EBV-HLH 患者进行了回顾性分析。在一部分患者中完成了额外的免疫研究。

结果

我们报告了 15 名被诊断为 T 细胞-EBV-HLH 的患者（10 个月至 19 岁）。9 名患者是西班牙裔，大多数没有原发性 HLH (p-HLH) 基因缺陷。T 细胞-EBV-HLH 中的可溶性白细胞介素 2 受体水平显着高于其他形式的继发性 HLH，但与 p-HLH 相当，并且与就诊时的疾病严重程度相关。尽管 p-HLH 检查呈阴性，但大多数患者的自然杀伤细胞功能下降。根据疾病严重程度，初始治疗包括地塞米松或地塞米松和依托泊苷。难治性患者采用混合方案进行管理，其中包括以下一种或多种疗法：联合化疗、阿仑单抗、依帕单抗和纳武单抗。利妥昔单抗并未明显降低大多数患者的 EBV 病毒血症。非危重患者对免疫抑制治疗反应良好，无需接受异基因造血干细胞移植（HSCT）即可长期存活。阿仑单抗导致三名患者中的两名出现炎症。三名患者接受了异基因 HSCT，其中一名患者出现疾病复发。在中位随访 3 年时，15 名患者中有 10 名存活。

结论

T 细胞-EBV-HLH 发生在美国的非亚洲人群中，尤其是西班牙裔人群。