从肝肿大到 POEMS 综合征:病例报告

IF 1.2 Q4 GASTROENTEROLOGY & HEPATOLOGY Hepatology Forum Pub Date : 2024-01-16 eCollection Date: 2024-01-01 DOI:10.14744/hf.2022.2022.0035
Yavuz Ozden, Sebnem Gursoy
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引用次数: 0

摘要

我们介绍了一例来自土耳其的 POEMS 综合征病例,这是一种罕见的、由浆细胞异常引起的多系统疾病。POEMS 是一个首字母缩写词,代表其主要特征:周围神经病变;器官肿大;内分泌病变;单克隆浆细胞增殖障碍;皮肤改变。据估计,该综合征的发病率为每 10 万人中有 0.3 人患病,通常出现在患者的第五或第六个十年。进行性周围神经病变是该综合征最突出的症状。为确保准确诊断,全面的病史、体格检查和综合诊断评估至关重要。这些评估应包括血清免疫电泳、血清细胞因子和生长因子、骨骼检查和骨髓活检。POEMS 综合征的早期识别和治疗对于防止病情恶化和优化临床疗效至关重要。
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From hepatomegaly to POEMS syndrome: A case report.

We present a case of POEMS syndrome from Turkiye, a rare, multisystemic condition resulting from plasma cell dyscrasia. POEMS is an acronym representing its cardinal features: Peripheral neuropathy; Organomegaly; Endocrinopathy; Monoclonal plasma-cell proliferative disorder; and Skin changes. The syndrome has an estimated prevalence of 0.3 per 100,000 individuals and typically manifests in the fifth or sixth decade of life. Progressive peripheral neuropathy is the syndrome's most prominent symptom. To ensure an accurate diagnosis, a thorough medical history, physical examination, and comprehensive diagnostic evaluations are essential. These evaluations should include serum immunoelectrophoresis, serum cytokines, and growth factors, a skeletal survey, and a bone marrow biopsy. Early recognition and treatment of POEMS syndrome are crucial to prevent debilitating progression and to optimize clinical outcomes.

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