Liu Yuanyuan, Lu Weiping, Zhang Hong, Li Xiang
Doctor, Department of Endocrinology, The Huai-an First Affiliated Hospital of Nanjing Medical University, Jiangsu, China.
Doctor, Clinical Laboratory, Huai'an Hospital Affiliated of Xuzhou Medical College, Jiangsu, China
Scott Med J. 2015 Aug;60(3):e5-8. doi: 10.1177/0036933015577538. Epub 2015 Mar 27.
Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome is a rare paraneoplastic syndrome from a plasma cell dyscrasia, which most commonly presents with peripheral neuropathy. We report a 30-year-old female with blurred vision and vision loss as reasons for her initial treatment; the patient was diagnosed with bilateral opticneuritis. Five months later, the patient started to have lower extremity numbness and fatigue. Initial laboratory tests did not reveal positive monoclonal immunoglobulin. Therefore, simple peripheral neuropathy was considered for diagnosis, and the patient was treated in the Department of Neurology in our hospital; however, the symptom was not alleviated. Seven months later, the patient showed symptoms of cough, dyspnea, along with rough skin, pigmentation and drooping of both feet. Chest CT revealed bronchiectasis and infection of lungs, destruction of thoracic bones and ribs. Further examination indicated that the patient had splenomegaly, hypothyroidism and monoclonal IgA-λ chain zone, which led to a clear diagnosis of Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin changes syndrome.
多神经病、器官肿大、内分泌病、M蛋白血症和皮肤改变综合征是一种由浆细胞发育异常引起的罕见副肿瘤综合征,最常见的表现是周围神经病变。我们报告一名30岁女性,最初因视力模糊和视力丧失接受治疗;该患者被诊断为双侧视神经炎。五个月后,患者开始出现下肢麻木和乏力。初始实验室检查未发现阳性单克隆免疫球蛋白。因此,诊断考虑为单纯性周围神经病变,患者在我院神经内科接受治疗;然而,症状并未缓解。七个月后,患者出现咳嗽、呼吸困难症状,同时伴有皮肤粗糙、色素沉着和双足下垂。胸部CT显示支气管扩张和肺部感染、胸椎和肋骨破坏。进一步检查表明患者有脾肿大、甲状腺功能减退和单克隆IgA-λ链区,从而明确诊断为多神经病、器官肿大、内分泌病、M蛋白血症和皮肤改变综合征。