临床神经病学杂志2018，Vol.31Issue(2)：107-110,4.
POEMS综合征的临床特征及诊疗分析
Clinical manifestations and analysis of diagnosis and treatment of POEMS syndrome
摘要
Abstract
Objective To observe the clinical manifestations of POEMS syndrome, and to improve the early diagnosis and prognosis.Methods The clinical data of 61 POEMS syndrome patients were analyzed retrospectively. Results Among the 61 patients,there were 27 males and 34 females with an average age of 47.2 years.The average time from the primary admission to the confirmed diagnosis was 22.5 months.All the patients(100%)had peripheral neuropathy by various degrees,the majority showing the sensory dysfunction of lower limbs.Fifty-two cases (85.2%)were identified with organomegaly by imaging techniques,most presented with the enlargement of spleen and lymph nodes.Fourty-five cases(73.8%)were accompanied with endocrinopathy, reflected by diabetics along with the hypofunction of the pituitary, gonad, thyroid and the adrenal glands.Fifty cases(82.0%)were with positive serum M protein.Twenty-seven cases(44.3%)manifested evident skin changes including hair growth along the limbs and trunk, skin pigmentation and skin keratinization of the feet.In this group of patients, 59 patients mainly use dexamethasone treatment,some patients with horses flange or cyclophosphamide,lenalidomide treatment. The other 2 patients were treated by autologous stem cell transplantation therapy.The clinical outcome of patients with diagnosis time ≤10 months was significantly better than that of patients with diagnosis time 10 to 20 months and≥20 months(all P<0.05).Patients with diagnosis time ≥20 months had the worst clinical outcomes, with the highest death rate(all P<0.05).Conclusions The clinical manifestations of POEMS syndrome are complex, especially the peripheral nerve damage symptoms and merging other organs or systems.M protein detection is needed in order to make clear diagnosis.Early diagnosis and effective treatment for patients can get better prognosis.关键词
POEMS综合征/临床特征/诊断/预后Key words
POEMS syndrome/clinical features/diagnosis/prognosis分类
医药卫生引用本文复制引用
葛义俊,戴映,高建国..POEMS综合征的临床特征及诊疗分析[J].临床神经病学杂志,2018,31(2):107-110,4.基金项目
安徽省高校自然科学研究重点项目(KJ2016A322) （KJ2016A322）
