Hara Daisuke, Akiyama Hisanao, Nukui Saki, Shimizu Takahiro, Hoshikawa Masahiro, Hasegawa Yasuhiro
Department of Internal Medicine, Division of Neurology Department of Diagnostic Pathology, Division of Diagnostic Pathology, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.
Medicine (Baltimore). 2017 Oct;96(41):e8188. doi: 10.1097/MD.0000000000008188.
We report a case of successful diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome based on monoclonality that was confirmed by an osteosclerotic lesion biopsy in a patient without pathognomonic symptoms or monoclonal gammopathy, probably because of comorbidities, which included systemic lupus erythematosus, rheumatoid arthritis, and Sjögren syndrome.
A 57-year-old woman presented with an approximately 2-year history of numbness in the toes that had gradually spread, along with muscle weakness in both arms and legs. She had been receiving immunosuppressant and corticosteroid therapy since being diagnosed with systemic lupus erythematosus and Sjögren syndrome at the age of 31 years and rheumatoid arthritis at the age of 44 years. Neurological examination revealed predominantly distal hypoesthesia and weakness in a typical stocking-and-glove pattern. Immunoelectrophoresis revealed elevated polyclonal immunoglobulin, which was attributed to her known underlying disease.
Biopsy of an osteosclerotic lesion confirmed proliferation of monoclonal plasma cells, leading to a diagnosis of POEMS syndrome.
Lenalidomide therapy was started after the diagnosis and the patient had a favorable outcome.
Osteosclerotic lesion biopsy can be useful for diagnosis of POEMS syndrome in difficult cases.
我们报告了一例成功诊断POEMS（多发性神经病、脏器肿大、内分泌病、单克隆丙种球蛋白病和皮肤改变）综合征的病例，该诊断基于单克隆性，通过对一名无典型症状或单克隆丙种球蛋白病患者的骨硬化性病变活检得以证实，这可能是由于其合并系统性红斑狼疮、类风湿关节炎和干燥综合征等合并症所致。
一名57岁女性，有大约2年的脚趾麻木病史，且逐渐蔓延，同时伴有双臂和双腿肌肉无力。她31岁被诊断为系统性红斑狼疮和干燥综合征，44岁被诊断为类风湿关节炎，此后一直在接受免疫抑制剂和皮质类固醇治疗。神经系统检查显示主要为典型的手套-袜套样分布的远端感觉减退和无力。免疫电泳显示多克隆免疫球蛋白升高，这归因于其已知的基础疾病。
骨硬化性病变活检证实单克隆浆细胞增殖，从而诊断为POEMS综合征。
诊断后开始使用来那度胺治疗，患者预后良好。
骨硬化性病变活检对疑难病例的POEMS综合征诊断可能有用。
