Wei C, Zhao D Q, Zhang Y, Wang W, Zhang W, Zhou D B
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2022 Apr 14;43(4):311-315. doi: 10.3760/cma.j.issn.0253-2727.2022.04.008.
To evaluate the clinical characteristics, treatment, and prognosis of patients with paraneoplastic neurological syndrome (PNS) associated with lymphoma. 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. 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. 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.
评估与淋巴瘤相关的副肿瘤性神经系统综合征（PNS）患者的临床特征、治疗及预后。回顾性分析2012年1月至2021年5月在北京协和医院接受治疗的11例淋巴瘤合并PNS患者的临床资料。11例患者中（8例男性，3例女性），发病年龄中位数为61岁（范围33 - 78岁）。10例患者PNS症状先于淋巴瘤出现。从PNS发病到淋巴瘤诊断的中位时间为4个月。11例患者中，1例为霍奇金淋巴瘤，8例为B细胞非霍奇金淋巴瘤，2例为外周T细胞淋巴瘤。7例患者检测了肿瘤神经抗体，其中2例阳性（1例抗Ma2抗体阳性，1例抗Yo抗体阳性）。11例患者中，3例PNS症状位于中枢神经系统，4例位于外周神经系统，3例位于肌肉。11例患者中有8例在淋巴瘤诊断前接受了以糖皮质激素为基础的免疫抑制治疗。中枢神经系统受累及皮肌炎患者对糖皮质激素反应良好，而外周神经病变患者获益不明显。11例患者均在淋巴瘤诊断后接受化疗。评估了9例患者的化疗疗效，7例完全缓解，1例病情稳定，1例病情进展。完全缓解患者的PNS症状几乎完全恢复。中位随访时间为42个月（范围4 - 95个月）。随访结束时，11例患者中6例存活，3例失访，2例死亡。全组中位总生存期未达到。PNS可累及神经系统的各个部位，并可与不同类型的淋巴瘤相关。通过早期诊断和治疗，大多数淋巴瘤完全缓解的患者PNS症状可改善。
