以肝脾肿大为首发表现的POEMS综合征2例报告

张野, 王文清, 李璟, 白芡蓉, 李佳玉, 成艳, 房苗苗, 高娜娜, 黄长形

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临床肝胆病杂志 ›› 2025, Vol. 41 ›› Issue (1) : 127-132. DOI: 10.12449/JCH250119
病例报告

以肝脾肿大为首发表现的POEMS综合征2例报告

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POEMS syndrome with hepatosplenomegaly as the initial manifestation: A report of two cases

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摘要

POEMS综合征是一种与浆细胞病有关、累及多系统的罕见病,临床表现多样。现报道2例以肝脾肿大为首发表现的POEMS综合征病例,病程中患者出现下肢无力、肝脾和淋巴结肿大、腹水、甲状腺功能减退、M蛋白阳性、皮肤色深,18F-FDG PET-CT显像发现以溶骨性改变为主的骨病变,合并浆细胞瘤,血清血管内皮生长因子水平升高,最终诊断为POEMS综合征,经免疫调节治疗后症状缓解。

Abstract

POEMS syndrome is a rare condition associated with plasma cell disorders, and it often involves multiple systems and has diverse clinical manifestations. This article reports two cases of POEMS syndrome with hepatosplenomegaly as the initial manifestation. During the course of the disease, the patients presented with lower limb weakness, hepatosplenomegaly, lymph node enlargement, ascites, hypothyroidism, positive M protein, and skin hyperpigmentation, and 18F-FDG PET-CT imaging revealed bone lesions mainly characterized by osteolytic changes and plasma cell tumors. There was an increase in the serum level of vascular endothelial growth factor. The patients were finally diagnosed with POEMS syndrome, and the symptoms were relieved after immunomodulatory treatment.

关键词

POEMS综合征 / 肝肿大 / 脾肿大 / 诊断

Key words

POEMS Syndrome / Hepatomegaly / Splenomegaly / Diagnosis

引用本文

导出引用
张野 , 王文清 , 李璟 , . 以肝脾肿大为首发表现的POEMS综合征2例报告. 临床肝胆病杂志. 2025, 41(1): 127-132 https://doi.org/10.12449/JCH250119
Ye ZHANG, Wenqing WANG, Jing LI, et al. POEMS syndrome with hepatosplenomegaly as the initial manifestation: A report of two cases[J]. Journal of Clinical Hepatol. 2025, 41(1): 127-132 https://doi.org/10.12449/JCH250119

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作者贡献声明

张野负责临床病例诊疗,资料收集,论文撰写;李璟、白芡蓉、李佳玉、成艳、房苗苗、高娜娜负责临床病例诊疗及资料收集;王文清、黄长形负责临床病例诊疗,研究指导,论文审核。

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