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POEMS综合征长期误诊并文献复习

         

摘要

Objective To explore the clinical characteristics and diagnosis criteria of POEMS syndrome, so as to re-duce the misdiagnosis and mistherapy rates. Methods Clinical data of one long time misdiagnosed case of POEMS syndrome was analyzed retrospectively. Meanwhile, the related literature was reviewed. Results The patient was admitted for intermit-tent tachypnea for over 2 years, acroanesthesia of lower limbs and costopathy for 9 months, skin itching for 7 months, limping for 3 months. At first, he was diagnosed as having diabetes mellitus, tuberculous pleuritis by laboratory tests, CT, bone scan-ning and pleural pathology in other hospitals. Then he was diagnosed as having chronic urticaria, skin pruritus for itchy skin, and metastatic adenocarcinoma ( to be excluded) for repeated hydrothorax. Finally, the patient was diagnosed as having PO-EMS syndrome in our hospital by the combination of PET-CT, bone lesions biopsy, bone marrow aspiration and immunohisto-chemisty, and underwent chemotherapy and radiotherapy, and the patient was in a remission. Conclusion POEMS syndrome is a rare, independent monoclonal plasma cell disorder, which has the clinical manifestations relaled to many systems and the therapeutic methods including chemotherapy and radiotherapy. Strengthening the awareness of this disease is helpful to avoid the misdiagnosis and mistherapy.%目的:探讨POEMS综合征的临床特点及诊断要点,以减少误诊误治。方法回顾分析1例长期误诊的POEMS综合征患者的临床资料,并复习相关文献。结果本例因间断气促2年余,双下肢麻木并发现肋骨病变9个月,全身皮肤瘙痒7个月,跛行3个月入院。曾在外院行实验室检查及CT、全身骨扫描、胸腔穿刺抽液等诊断为糖尿病、结核性胸膜炎;后患者出现全身皮肤瘙痒又诊断为慢性荨麻疹及皮肤瘙痒症,胸腔积液反复出现又诊断为转移性腺癌待排等。此次入院后行PET-CT、骨病灶穿刺、骨髓穿刺及免疫组织化学等检查明确诊断为POEMS综合征,并接受全身化疗及局部放疗,病情明显缓解。结论 POEMS综合征是一种罕见的独立的克隆性浆细胞疾病,临床表现涉及全身多个系统,治疗以化疗、放疗为主;加强对此病的认识,可减少或避免误诊误治。

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