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非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的临床特征及治疗结果

[Clinical features and treatment outcome of patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis].

作者信息

Sun S R, Wu M, Wulipan Fulati, Shen L, Ma J X, Chen P P, Hu Y W, Zhang H D, Xie Y H

机构信息

Department of Hematology, Huadong Hospital, Fudan University, Shanghai 200040, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2021 Apr 14;42(4):324-331. doi: 10.3760/cma.j.issn.0253-2727.2021.04.010.

Abstract

To investigate the clinical features and effect of prognostic factors in patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. 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. 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: =0041, PFS: =0.015) , ECOG score ≥ 3 (OS: =0.031, PFS: =0.030) , hematopoietic stem cell transplantation (OS: =0.005, PFS: =0.040) , lymphadenopathy (OS: =0.007, PFS: =0.012) , and splenomegaly (OS: =0.276, PFS: =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. 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.

摘要

探讨不同病理类型的非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的临床特征及预后因素的影响。我们收集并分析了2013年3月至2020年5月在华东医院接受治疗的89例非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的临床资料。通过对数秩检验和Cox多因素分析对数据进行分析。89例患者的中位总生存时间为10.2个月。B细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者未达到中位总生存时间。T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症和NK细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症的中位总生存时间分别为10.2个月和3.0个月。非霍奇金淋巴瘤的病理类型(总生存:=0.041,无进展生存:=0.015)、东部肿瘤协作组(ECOG)评分≥3(总生存:=0.031,无进展生存:=0.030)、造血干细胞移植(总生存:=0.005,无进展生存:=0.040)、淋巴结病(总生存:=0.007,无进展生存:=0.012)和脾肿大(总生存:=0.276,无进展生存:=0.324)与非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的总生存和无进展生存相关。脾切除术可改善淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的预后,尤其是T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者。不同病理类型的非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者的临床特征相似,但总生存率和预后因素的影响不同。我们建议,非霍奇金淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者应接受联合化疗以上的治疗。为提高患者的预后和生存率,B细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症和NK细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者急需进行造血干细胞移植。此外,T细胞淋巴瘤相关噬血细胞性淋巴组织细胞增生症患者应考虑行脾切除术。

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