爱泼斯坦-巴尔病毒阳性淋巴瘤相关噬血细胞综合征：一项对51例患者的回顾性单中心研究。
Epstein-Barr Virus-Positive Lymphoma-Associated Hemophagocytic Syndrome: A Retrospective, Single-Center Study of 51 Patients.
作者信息
Zhao Ailin, Yang Jinrong, Li Meng, Li Linfeng, Gan Xinai, Wang Jie, Li He, Shen Kai, Yang Yunfan, Niu Ting
机构信息
Department of Hematology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
出版信息
Front Immunol. 2022 Apr 11;13:882589. doi: 10.3389/fimmu.2022.882589. eCollection 2022.
PURPOSE
To investigate clinical characteristics, management, and prognosis of Epstein-Barr virus (EBV)-positive lymphoma-associated hemophagocytic syndrome (LAHS) patients in real-world practice.
METHODS
This was a retrospective, single-center cohort study. EBV-positive LAHS patients diagnosed from January 2010 to December 2021 in our center were enrolled. Clinical characteristics, treatment, overall response rate (ORR), and overall survival (OS) were investigated. Univariate and multivariate analysis of potential factors were conducted.
RESULTS
Of the 51 patients, 44 were T/NK cell lymphoma; five were B cell lymphoma; two were Hodgkin lymphoma. EBV-positive T/NK cell LAHS patients were significantly younger and showed lower fibrinogen levels and C-reactive protein levels than EBV-positive B cell LAHS patients (=0.033, =0.000, and =0.004, respectively). Combined treatment of anti-hemophagocytic lymphohistiocytosis (HLH) and anti-lymphoma treatment was conducted in 24 patients; anti-HLH treatment was conducted in 18 patients; anti-lymphoma treatment was conducted in three patients; glucocorticoid treatment was conducted in one patient. ORR was 47.8%, and the median OS was 61 (95% confidence interval 47.9-74.1) days for overall patients. Patients who received anti-HLH treatment and turned to anti-lymphoma treatment early displayed higher ORR and OS than those of anti-HLH patients (=0.103, and =0.003, respectively). Elevated alanine aminotransferase level was the independent risk factor of EBV-positive LAHS prognosis.
CONCLUSIONS
Prognosis of EBV-positive LAHS patients was poor. Anti-lymphoma treatment should be initiated as soon as HLH was rapidly controlled.
目的
探讨在实际临床实践中，爱泼斯坦-巴尔病毒（EBV）阳性淋巴瘤相关噬血细胞综合征（LAHS）患者的临床特征、治疗方法及预后情况。
方法
这是一项回顾性单中心队列研究。纳入了2010年1月至2021年12月在本中心诊断的EBV阳性LAHS患者。对其临床特征、治疗情况、总缓解率（ORR）和总生存期（OS）进行了研究。对潜在因素进行了单因素和多因素分析。
结果
51例患者中，44例为T/NK细胞淋巴瘤；5例为B细胞淋巴瘤；2例为霍奇金淋巴瘤。EBV阳性T/NK细胞LAHS患者比EBV阳性B细胞LAHS患者明显年轻，纤维蛋白原水平和C反应蛋白水平更低（分别为P = 0.033、P = 0.000和P = 0.004）。24例患者接受了抗噬血细胞性淋巴组织细胞增生症（HLH）和抗淋巴瘤联合治疗；18例患者接受了抗HLH治疗；3例患者接受了抗淋巴瘤治疗；1例患者接受了糖皮质激素治疗。总体患者的ORR为47.8%，中位OS为61天（95%置信区间47.9 - 74.1）。早期接受抗HLH治疗并转为抗淋巴瘤治疗的患者比单纯抗HLH治疗的患者显示出更高的ORR和OS（分别为P = 0.103和P = 0.003）。谷丙转氨酶水平升高是EBV阳性LAHS预后的独立危险因素。
结论
EBV阳性LAHS患者预后较差。一旦HLH得到快速控制，应尽早开始抗淋巴瘤治疗。
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