POEMS综合征20例临床特征分析

Analysis of Clinical Features on 20 Cases of POEMS Syndrome

目的分析总结POEMS综合征临床特征,以提高医师认识水平,减少误诊。方法回顾性分析2005年1月—2012年4月我院收治的20例POEMS综合征患者的临床表现、辅助检查、治疗及预后,并复习相关文献。结果 20例患者均有多发性周围神经病变和皮肤改变;单克隆浆细胞增生18例,血清M蛋白阳性率为90.0%,免疫固定电泳阳性率高达100.0%(17/17),12例行骨髓穿刺,骨髓细胞涂片浆细胞平均比例为3.2%;脏器肿大16例,内分泌异常19例;水负荷增多19例。本组肾功能受累较常见,其中β2微球蛋白增高12例、肌酐增高4例。其他特征肺动脉压增高8例、贫血10例、血小板增多5例、多发骨骼异常2例及顽固性腹痛1例。本组误诊率为95.0%,误诊为格林巴利综合征8例,甲状腺功能减退症4例,其他内科疾病7例。病死率为5.0%(1/20)。结论 POEMS综合征以各种症状为首发,临床表现复杂,误诊率极高,导致病情发展,临床医师应加深认识,对疑诊患者应进行细致的检查以避免误诊。

Objective To analyze and summarize the clinical features of POEMS syndrome in order to improve the recognition of POEMS syndrome and reduce misdiagnosis. Methods Clinical manifestations, auxiliary examinations, treatment and prognosis of 20 patients with POEMS syndrome admitted to our hospital were retrospectively analyzed and related literatures were also reviewed. Results All 20 patients had polyneuropathy and skin changes. 18 cases had monoclonal plasmacytosis. The positive rate of serum M protein was 90. 0% and the positive rate of immunofixation was up to 100. 0% （ 17/17 ）. 12 cases were given bone marrow puncture and the average proportion of plasmocyte in bone marrow smear was 3.2%. 16 cases had swell- ing organs, 19 cases had endocrine abnormality and 19 cases had increased water load. Renal insufficiency was common. 12 cases had increased β2 - microglobulin and 4 cases had increased creatinine. Other clinical features were shown as follows ： 8 cases had increased pulmonary artery pressure, l0 cases had anemia, 5 cases had thrombocythemia, 2 cases had multiple skeletal a- nomalies and one case had refractory abdominal pain. The misdiagnosis rate was 95.0% , of which 8 cases were misdiagnosed as Guillain - Barre Syndrome, 4 cases were misdiagnosed as hypothyroidism and 7 cases were misdiagnosed as other medical diseases. The mortality rate was 5.0% （1/20）. Conclusion POEMS syndrome has various first symptoms with complicated clinical features and could be easily misdiagnosed, causing development of the disease. Clinical doctors should deepen understanding of the disease and perform careful examinations for suspected patients to avoid misdiagnosis.