淋巴瘤合并神经系统副肿瘤综合征11例临床分析

Clinical analysis of 11 cases of lymphoma complicated with paraneoplastic neurological syndrome

目的探讨淋巴瘤合并神经系统副肿瘤综合征(PNS)患者的临床特点、治疗及预后。方法回顾性分析2012年1月至2021年5月于北京协和医院诊治的11例淋巴瘤合并PNS患者的临床资料。结果11例患者中,男8例,女3例,中位发病年龄为62(16~74)岁。10例患者PNS症状先于淋巴瘤出现,出现PNS症状至诊断淋巴瘤的中位时间为4个月。11例患者中包括霍奇金淋巴瘤1例、B细胞型非霍奇金淋巴瘤8例、外周T细胞淋巴瘤2例。7例患者进行了肿瘤神经抗体的检测,2例阳性,分别为抗Ma2抗体和抗Yo抗体。11例患者中,PNS症状定位于中枢神经系统者3例,定位于周围神经系统者4例,定位于肌肉者3例。11例患者中8例在诊断淋巴瘤前采用以糖皮质激素为主的免疫抑制治疗,中枢神经系统病变及皮肌炎患者对免疫抑制治疗反应良好,周围神经病变患者激素治疗获益不明显。11例患者在诊断淋巴瘤后均采用针对淋巴瘤的化疗。其中9例行疗效评估,7例为完全缓解,1例为疾病稳定,1例为疾病进展。淋巴瘤获得完全缓解的患者PNS症状均获得极大改善。中位随访时间为42(4~95)个月。截至随访终止日期,11例患者中6例存活,3例失访,2例死亡,整组患者中位OS时间未达到。结论PNS可累及神经系统的各个组成部分,可并发于各种类型的淋巴瘤。早期通过化疗达到肿瘤的完全缓解可使多数患者PNS症状改善。

Objective To evaluate the clinical characteristics,treatment,and prognosis of patients with paraneoplastic neurological syndrome(PNS)associated with lymphoma.Methods Between January 2012 and May 2021,the clinical data of 11 patients with lymphoma complicated with PNS treated at Peking Union Medical College Hospital were retrospectively reviewed.Results Among the 11 patients(8 male and 3 female),the median onset age was 61(range,33-78)years.The symptoms of PNS preceded lymphoma in 10 patients.The median time from the onset of PNS to the diagnosis of lymphoma was 4 months.Of the 11 patients,one had Hodgkin’s lymphoma,8 had B-cell non-Hodgkin’s lymphoma,and 2 had peripheral T-cell lymphoma.Seven patients were evaluated for onconeural antibody,of whom 2 were positive(1 for anti-Ma2 antibody and 1 for anti-Yo antibody).Of the 11 patients,the PNS symptoms of 3 patients were located in the central nervous system,4 were located in the peripheral nervous system,and 3 were located in the muscle.Eight of the 11 patients were treated with glucocorticoid-based immunosuppressive therapy before the diagnosis of lymphoma.Patients with central nervous system involvement and dermatomyositis responded well to glucocorticoid,whereas patients with peripheral neuropathy did not significantly benefit.All 11 patients were treated with chemotherapy after the diagnosis of lymphoma.The efficacy of chemotherapy was assessed in 9 patients,7 cases achieved complete remission,1 case was evaluated as stable disease,and 1 case was evaluated as disease progression.The PNS symptoms of the patients who achieved complete response were almost completely recovered.The median follow-up time was 42(range,4-95)months.At the end of the follow-up period,6 of the 11 patients survived,3 were lost to follow-up,and 2 died.The median overall survival of the whole group was not reached.Conclusions PNS can involve various parts of the nervous system and can be associated with different types of lymphoma.Through early diagnosis and treatment,the PNS symptoms could improve in most patients who achieve complete remission of lymphoma.