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

doi:10.12449/JCH250119

以肝脾肿大为首发表现的POEMS综合征2例报告

DOI: 10.12449/JCH250119

张野^1a,
王文清^1b,
李璟^1a,
白芡蓉^1c,
李佳玉^1a,
成艳^1a,
房苗苗²,
高娜娜³,
黄长形^1a, , ,

1a.
空军军医大学第二附属医院（第四军医大学唐都医院）传染病科，西安 710038
1b.
空军军医大学第二附属医院（第四军医大学唐都医院）血液内科，西安 710038
1c.
空军军医大学第二附属医院（第四军医大学唐都医院）核医学科，西安 710038
2.
西安市临潼区中医院肝病科，西安 710699
3.
西安市中医医院肝病科，西安 710021

基金项目:

陕西省传染疾病临床医学研究中心 (2021LCZX-09)

伦理学声明： 本例报告已获得患者及其家属知情同意。

利益冲突声明：本文不存在任何利益冲突。

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。

详细信息

通信作者:
黄长形， 13572133011@163.com （ORCID： 0000-0001-5511-2999）

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出版历程
- 收稿日期: 2024-05-30
- 录用日期: 2024-06-20
- 出版日期: 2025-01-25

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

1a.
Department of Infectious Diseases The Second Affiliated Hospital of Air Force Medical University （Tangdu Hospital，Fourth Military Medical University），Xi’an 710038，China
1b.
Department of Hematology The Second Affiliated Hospital of Air Force Medical University （Tangdu Hospital，Fourth Military Medical University），Xi’an 710038，China
1c.
Department of Nuclear Medicine The Second Affiliated Hospital of Air Force Medical University （Tangdu Hospital，Fourth Military Medical University），Xi’an 710038，China
2.
Department of Hepatology，Lintong Hospital of Traditional Chinese Medicine，Xi’an 710699，China
3.
Department of Hepatology，Xi’an Hospital of Traditional Chinese Medicine，Xi’an 710021，China

Research funding:

Clinical Research Center for Infectious Diseases of Shaanxi Province (2021LCZX-09)

More Information

Corresponding author: HUANG Changxing， 13572133011@163.com （ORCID： 0000-0001-5511-2999）

摘要

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。
- POEMS综合征 /
- 肝肿大 /
- 脾肿大 /
- 诊断
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.
- POEMS Syndrome /
- Hepatomegaly /
- Splenomegaly /
- Diagnosis

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注： a，骨髓活检见成堆成簇浆细胞（苏木素-吉姆萨-酸性品红染色，×40）；b，¹⁸F-FDG PET-CT示右侧髂骨骨质破坏伴葡萄糖代谢增高（绿色箭头）；c，¹⁸F-FDG PET-CT示腰4椎体骨质破坏伴葡萄糖代谢增高（绿色箭头）；d，¹⁸F-FDG PET-CT示脾大伴腹腔积液；e，¹⁸F-FDG PET-CT示腹膜后淋巴结（绿色箭头）。

图 1 病例1骨髓活检及¹⁸F-FDG PET-CT检查结果

Figure 1. The results of bone marrow biopsy and ¹⁸F-FDG PET-CT of case 1

下载: 全尺寸图片幻灯片

注： a，患者皮肤颜色暗沉发黑，体毛重；b，¹⁸F-FDG PET-CT示骨盆、双侧股骨、多个椎体及附件高密度影，部分代谢增高（绿色箭头）；c，¹⁸F-FDG PET-CT示腹腔积液、脾大，肝胃间隙淋巴结伴代谢增高（绿色箭头）。

图 2 病例2皮肤表现及¹⁸F-FDG PET-CT结果

Figure 2. The results of skin change and ¹⁸F-FDG PET-CT of case 2

下载: 全尺寸图片幻灯片

参考文献(12)

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