Kawanishi Kazunobu, Ohyama Yasuyo, Kanai Yoshitake, Hirase Tikara, Tanaka Hirokazu, Miyatake Junichi, Tatsumi Youichi, Ashida Takashi, Nakamine Hirokazu, Matsumura Itaru
Department of Internal Medicine, Kinki University, Japan.
Intern Med. 2012;51(15):2015-20. doi: 10.2169/internalmedicine.51.7457. Epub 2012 Aug 1.
Here we report the first case of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), who initially presented with peripheral neuropathy. Nerve conduction, cerebral spinal fluid studies and his clinical course were compatible with sub-acute demyelinating polyradiculoneuropathy. In addition, left cervical lymph node swelling was observed on admission. Diagnosis of PTCL-NOS was made by the histological, immunohistochemical, and Southern blot analyses on the biopsy specimen from the enlarged lymph node. Combination chemotherapy composed of cyclophosphamide, vincristine, doxorubicin and prednisolone (CHOP) was effective for polyneuropathy as well as for lymphoma. Several antibodies relating to paraneoplastic syndrome such as Ma1, Ma2, Amphiphysin, CV2, Ri, Yo and Hu were all negative. Because sural nerve biopsy performed prior to CHOP therapy revealed no infiltration of lymphoma cells, immune dysfunction mediated by some cytokine or unidentified autoantibody related to PTCL-NOS was thought to be involved in the polyradiculoneuropathy.
在此，我们报告首例最初表现为周围神经病变的外周T细胞淋巴瘤，非特指型（PTCL-NOS）。神经传导、脑脊液检查及患者的临床病程均符合亚急性脱髓鞘性多发性神经根神经病。此外，入院时观察到左侧颈部淋巴结肿大。通过对肿大淋巴结活检标本进行组织学、免疫组化及Southern印迹分析确诊为PTCL-NOS。由环磷酰胺、长春新碱、阿霉素和泼尼松龙组成的联合化疗（CHOP）对多发性神经病及淋巴瘤均有效。几种与副肿瘤综合征相关的抗体，如Ma1、Ma2、抗 amphiphysin 抗体、CV2、Ri、Yo和Hu均为阴性。因为在CHOP治疗前进行的腓肠神经活检未发现淋巴瘤细胞浸润，所以认为由某些细胞因子或与PTCL-NOS相关的未明自身抗体介导的免疫功能障碍与多发性神经根神经病有关。
