原发性中枢神经系统淋巴瘤62例临床病理学特征及预后

Clinical features and treatment outcomes in primary central nervous system lymphoma:a descriptive analysis of 62 patients

目的探讨原发性中枢神经系统淋巴瘤的临床特征、组织病理学特点、治疗方案及其与预后的关系。方法收集1998年1月至2012年1月期间广东省人民医院诊治的免疫功能正常的、有完整临床和随访资料、可进行系统性评估的原发性中枢神经系统淋巴瘤病例62例。采用Kaplan-Meier生存分析、Cox风险比例回归模型列出相关生存分析曲线,观察患者预后。结果62例患者中位发病年龄约为56岁,男女发病率为1.14∶1.00,临床首发症状多为颅内压升高以及局灶神经损害。54例(54/62,87.1%)患者为国际预后指数(IPI)0~2。组织学类型上,弥漫性大B细胞淋巴瘤占91.9%(57/62),T细胞淋巴瘤占6.4%(4/62),结外边缘区黏膜相关淋巴组织淋巴瘤占1.6%(1/62)。其中,32例(32/62,51.6%)可见颅内多发病灶,30例(30/62,48.4%)可见深部器官累及。19例(19/62,30.6%)患者乳酸脱氢酶(LDH)升高,38例(38/62,61.3%)Ki-67阳性指数≥90%。单因素生存分析显示,患者性别、年龄、体能状态评分、IPI、是否为单发病变以及有无深处器官侵犯、LDH有无升高、不同的治疗方法与患者的预后密切相关。Cox风险比例回归模型多因素分析显示,应用利妥昔单抗+大剂量的甲氨蝶呤+全脑放疗治疗组患者的预后好,差异具有统计学意义(P<0.05)。结论原发性中枢神经系统淋巴瘤进展快、预后差、临床表现复杂多样,应用利妥昔单抗+大剂量的甲氨蝶呤+全脑放疗治疗组中位总生存时间显著延长,可能是未来治疗方向之一。

Objective To assess clinical features and treatment outcomes in immunocompetent patients with primary central nervous system lymphoma(PCNSL).Methods Sixty-two patients with PCNSL who attended Guangdong General Hospital between January 1998 and January 2012 were included.Survival curves were estimated using Kaplan-Meier survival methodology and statistical significance of continuous was assessed via the Cox proportional hazard model.Results The median age of the patient cohort was 56 years,and the male to female ratio was 1.14∶1.00.The common presentations were increased intracranial pressure symptoms and neuron damage.Performance status of 54(54/62,87.1%)patients were the international prognostic index(IPI)0-2.Diffuse large B-cell lymphoma(57/62,91.9%)was most common,and the rest were T-cell lymphoma(4/62,6.4%)and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue(1/62,1.6%).In the series,32 patients(32/62,51.6%)had multiple lesions.Involvement of deep structures was found in 30(30/62,48.4%)patients.An elevated serum LDH level was detected in 19(19/62,30.6%)patients and the Ki-67 index was≥90%in 38(38/62,61.3%)patients.Univariate analysis showed patients who were female,age<60 years,had WHO Eastern Cooperative Oncology Group performance status grade 0-2,single lesion,absence of deep structures involvement and normal LDH level showed better 2-year survival rate and longer median survival time.Significance was only seen in the normal LDH level group.Multivariate Cox regression analysis revealed that radical surgery only and Rituximab+high-dose of methotrexate+whole brain radiation therapy(WBRT)were independent prognostic indicators in PCNSL patients(P<0.05).Conclusions PCNSL is a rare but aggressive tumor with poor prognosis.Patients treated with high-dose of methotrexate combining with rituximab,followed by WBRT have a better prognosis and longer survival time,and thus these could probably be a promising treatment.