原发中枢神经系统淋巴瘤与继发中枢神经系统淋巴瘤的临床特征比较

刘丽欣; 刘景华; 周凡

doi:10.3969/j.issn.2095-5227.2022.01.003

原发中枢神经系统淋巴瘤与继发中枢神经系统淋巴瘤的临床特征比较

Clinical features of primary versus secondary central nervous system lymphoma

摘要

摘要:
背景中枢神经系统淋巴瘤(central nervous system lymphoma，CNSL)分为原发中枢神经系统淋巴瘤(primary central nervous system lymphoma，PCNSL)和继发中枢神经系统淋巴瘤(secondary central nervous system lymphoma，SCNSL)，近年来发生率逐年增高，目前对于PCNSL和SCNSL各自的临床特征缺乏一定的认识。
目的通过分析PCNSL和SCNSL患者的临床资料，提高对PCNSL和SCNSL各自临床特征的认识。
方法回顾性分析北部战区总医院血液科2015年1月- 2020年12月收治的29例PCNSL患者和16例SCNSL患者的临床资料，分析两组患者的临床表现和诊治特点。
结果 CNSL患者占北部战区总医院同期收治的淋巴瘤患者(461例)的9.76%，PCNSL组与SCNSL组是否出现B组症状、初始乳酸脱氢酶(lactate dehydrogenase，LDH)水平、颅内病灶位置、结外浸润与否和细胞来源差异有统计学意义(P<0.05)，而性别、年龄、最高LDH水平、生发中心来源与否、CNS-IPI评分、IELSG评分、脑脊液指标、治疗疗效差异无统计学意义(P>0.05)。PCNSL组病理均为B细胞来源淋巴瘤，26例(89.7%)初始LDH升高，病灶类型方面15例(51.7%)为单纯脑实质型，14例(48.3%)为脑膜与脑实质并存型；SCNSL组病理类型中T细胞来源和B细胞来源比例相近，8例(50%)初始LDH升高，病灶类型方面5例(31.3%)为单纯脑膜型，9例(56.2%)为脑膜与脑实质并存型。PCNSL组治疗总反应率(62.1%)高于SCNSL组(43.7%)。
结论 PCNSL多数患者为B细胞来源，初始LDH升高，均可通过影像学检测到实质病灶，半数可累及脑膜，出现脑脊液蛋白升高；SCNSL可为T细胞和B细胞来源，半数患者初始LDH升高，多出现脑脊液蛋白升高，半数以上通过影像学检测到实质病灶，总治疗有效率低于PCNSL。

Abstract:
Background Central nervous system lymphoma (CNSL) can be divided into primary central nervous system lymphoma (PCNSL) and secondary nervous system lymphoma (SCNSL). The incidence of CNSL is increasing in recent years, yet there is still a lack of analysis of their unique clinical characteristics.
Objective To evaluate the clinical characteristics of PCNSL and SCNSL by previous medical records, so as to improve understandings of PCNSL and SCNSL.
Methods From January 2015 to December 2020, 29 cases with PCNSL and 16 cases with SCNSL were diagnosed in the department of hematology of Northern Theater General Hospital, their clinical manifestations and diagnostic characteristics were analyzed.
Results CNSL accounted for 9.76% of 461 cases of lymphoma treated in our hospital during this period. There were significant differences in group B symptoms, initial LDH level, intracranial focus location, extramedullary infiltration and cell source between the two groups (P<0.05), however, no significant difference was found in sex, age, highest LDH level, origin of germinal center, CNS-IPI score, IELSG score, cerebrospinal fluid index or therapeutic effect (P>0.05). Pathological results showed that all patients in PCNSL group had B-cell-derived lymphoma. There were 26 cases (89.7%) with elevated initial LDH, 15 cases (51.7%) were simple brain parenchyma type, and 14 cases (48.3%) were meningeal and cerebral parenchyma coexisting type. In SCNSL group, patients with T cells or B cells showed similar proportion. The initial LDH increased in 8 patients (50.0%), simple meningeal type was found in 5 cases (31.3%), and 9 cases (56.2%) were meningeal and brain parenchyma type. The total response rate of PCNSL was higher than that of SCNSL (62.1% vs 43.7%).
Conclusion Most of the patients with PCNSL are B cells with increasing initial LDH. The parenchyma lesions can be detected by imaging, half of them are involved in the meninges, and the cerebrospinal fluid protein increases. SCNSL can be derived from T cells and B cells, the initial LDH of half of the patients increases, so does the cerebrospinal fluid protein. More than half of the parenchyma lesions are detected by imaging, and the total response rate of treatment is lower than that of PCNSL.

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