Objective: To investigate the clinical features and effect of prognostic factors in patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. Methods: We collected and analyzed the clinical data of 89 patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis who were treated at Huadong Hospital from March 2013 to May 2020. The data were analyzed via log-rank and Cox multivariate analyses. Results: The median overall survival time of the 89 cases was 10.2 months. Patients with B-cell lymphoma-associated hemophagocytic lymphohistiocytosis did not reach the median overall survival time. The median overall survival times of T-cell lymphoma-associated hemophagocytic lymphohistiocytosis and NK-cell lymphoma-associated hemophagocytic lymphohistiocytosis were 10.2 and 3.0 months, respectively. The pathological type of non-Hodgkin lymphoma (OS: P=0041, PFS: P=0.015) , ECOG score ≥ 3 (OS: P=0.031, PFS: P=0.030) , hematopoietic stem cell transplantation (OS: P=0.005, PFS: P=0.040) , lymphadenopathy (OS: P=0.007, PFS: P=0.012) , and splenomegaly (OS: P=0.276, PFS: P=0.324) were related to the overall survival and progression-free survival of patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. Splenectomy could improve the prognosis of patients with lymphoma-associated hemophagocytic lymphohistiocytosis, especially T-cell lymphoma-associated hemophagocytic lymphohistiocytosis. Conclusion: The clinical characteristics of patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis were similar but were different in the overall survival rate and the effect of prognostic factors. We suggested that patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis should receive more than combined chemotherapy. To improve the prognosis and survival rate of patients, those with B-cell lymphoma-associated hemophagocytic lymphohistiocytosis and NK-cell lymphoma-associated hemophagocytic lymphohistiocytosis promptly require hematopoietic stem cell transplantation. Moreover, patients with T-cell lymphoma-associated hemophagocytic lymphohistiocytosis should consider splenectomy.
目的： 回顾性分析不同病理类型非霍奇金淋巴瘤相关噬血细胞综合征（HLH）的临床特征及预后影响因素。 方法： 收集和分析2013年3月至2020年5月复旦大学附属华东医院确诊的89例非霍奇金淋巴瘤相关HLH患者的临床资料，应用Log-rank法对影响预后的因素进行单因素分析，应用Cox比例风险回归模型进行多因素分析。 结果： 89例淋巴瘤相关HLH患者中位生存时间10.2个月，B细胞淋巴瘤患者未达到中位生存时间，T细胞淋巴瘤患者中位生存时间10.2个月，NK细胞淋巴瘤患者中位生存时间为3个月，差异具有统计学意义（P=0.012）。非霍奇金淋巴瘤病理类型[总生存（OS）：P=0.041，无进展生存（PFS）：P=0.015]，美国东部肿瘤协作组评分（OS：P=0.031，PFS：P=0.030），是否行造血干细胞移植（OS：P=0.005，PFS：P=0.040），是否伴淋巴结肿大（OS：P=0.007，PFS：P=0.012），是否伴脾脏肿大（OS：P=0.276，PFS：P=0.324）是影响非霍奇金淋巴瘤相关HLH患者OS和PFS的因素。脾脏切除手术能够改善淋巴瘤相关HLH患者的生存，尤其是T细胞淋巴瘤相关HLH。 结论： 不同病理类型非霍奇金淋巴瘤相关HLH患者临床特点相似，但长期生存率和预后影响因素不同。非霍奇金淋巴瘤相关HLH不仅需要联合化疗，B细胞淋巴瘤和NK细胞淋巴瘤相关HLH患者需尽早进行造血干细胞移植，T细胞淋巴瘤相关HLH患者应考虑脾脏切除手术，以改善患者预后。.
Keywords: Hemophagocytic lymphohistiocytosis; Non-Hodgkin’s lymphoma; Prognosis; Survival analysis.