POEMS综合征长期误诊一例  

A case of Long-term misdiagnosis POEMS syndrome

作　　者：万芳 Wan Fang(Department of Hematology,The Third Afflicated Hospital of Gansu University of Traditional Chinese Medicine,the 1st Pepole′s Hospital of Baiyin,Baiyin 730900,China)

机构地区：[1]甘肃中医药大学第三附属医院,甘肃省白银市第一人民医院血液科,730900

出　　处：《中国综合临床》2020年第5期469-472,共4页Clinical Medicine of China

摘　　要：POEMS综合征是与浆细胞疾病有关的一种临床少见疾病,常累及多个系统,但不具有特异性。以典型"五联征",包括多发性周围神经病、器官肿大、内分泌障碍、M蛋白血症和皮肤病变为表现的患者临床诊断相对容易。具有典型表现的患者为极少数,因此在临床极易导致漏诊或误诊的出现。本例患者最初表现双下肢麻木、无力,后渐出现足底、背部疼痛,全身肿胀、皮肤色素沉着、阳痿等症状。曾先后到白银市第一人民医院脊柱外科、疼痛科住院。考虑吉兰-巴雷综合征、肌筋膜炎、腰椎间盘突出,予以对症治疗,效果不好。后逐渐出现胸闷、气短、胸腔积液,又在呼吸科住院,考虑慢性阻塞性肺疾病、肺动脉高压,期间检查发现浅表淋巴结肿大,予以行颈部淋巴结活检,考虑淋巴结浆细胞瘤。遂前往血液科住院,进一步完善骨髓穿刺涂片、免疫固定电泳、血轻链蛋白测定、髓样分化因子88、血管内皮细胞生长因子等检查,最终诊断为POEMS综合征,诊断后予以行全身化疗,病情明显缓解。POEMS syndrome is a rare clinical disease related to plasma cell disease.It often involves multiple systems,but it has no specificity.It is relatively easy to diagnose the typical"quintuple syndrome",including polyneuropathy,organomegaly,endocrine disorders,m-albuminemia and skin lesions.But in the process of clinical diagnosis and treatment,it is found that there are very few patients with typical manifestations,so it is easy to lead to missed diagnosis or misdiagnosis.In this case,numbness and weakness of both legs were found at first,then pain of foot and back,swelling of whole body,pigmentation of skin,impotence and other symptoms gradually appeared.He has been successively hospitalized in spine surgery and pain department of Baiyin first people′s hospital.Considering Guillain Barre syndrome,myofascitis and lumbar disc herniation,the effect of symptomatic treatment is not good.After that,he gradually developed chest tightness,shortness of breath and pleural effusion.In addition,he was hospitalized in the respiratory department.He considered chronic obstructive pulmonary disease and pulmonary hypertension.During the examination,he found superficial lymphadenopathy.He underwent neck lymph node biopsy and considered lymphadenoplasmacytoma.Then he went to the hematology department to be hospitalized,and further improved the bone marrow aspiration smear,immunofixation electrophoresis,blood light chain protein determination,myeloid differentiation factor 88(MyD88),vascular endothelial growth factor(VEGF)and other tests.Finally,he was diagnosed as poems syndrome.After diagnosis,he received systemic chemotherapy,and the condition was significantly relieved.

关 键 词：POEMS综合征 吉兰-巴雷综合征 血管内皮细胞生长因子 误诊 

分 类 号：R597[医药卫生—内科学]