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中华临床医师杂志(电子版) ›› 2017, Vol. 11 ›› Issue (17) : 2206 -2210. doi: 10.3877/cma.j.issn.1674-0785.2017.17.009

所属专题: 经典病例 文献

临床病例分析

表现为POEMS综合征的Castleman病伴肾损害一例并文献复习
赵酉璐1, 王文生2,(), 岑溪南2, 王茫桔2, 孙玉华2, 任汉云2   
  1. 1. 100034 北京,北京大学第一医院肾脏内科
    2. 100034 北京,北京大学第一医院血液内科
  • 收稿日期:2017-03-16 出版日期:2017-09-01
  • 通信作者: 王文生

POEMS syndrome and Castleman′s disease with renal involvement: a case report and review of the literature

Youlu Zhao1, Wensheng Wang2,(), Xinan Cen2, Mangju Wang2, Yuhua Sun2, Hanyun Ren2   

  1. 1. Renal Division, Peking University First Hospital, Beijing 100034, China
    2. Hematological Division, Department of Medicine, Peking University First Hospital, Beijing 100034, China
  • Received:2017-03-16 Published:2017-09-01
  • Corresponding author: Wensheng Wang
  • About author:
    Corresponding author: Wensheng Wang, Email:
引用本文:

赵酉璐, 王文生, 岑溪南, 王茫桔, 孙玉华, 任汉云. 表现为POEMS综合征的Castleman病伴肾损害一例并文献复习[J]. 中华临床医师杂志(电子版), 2017, 11(17): 2206-2210.

Youlu Zhao, Wensheng Wang, Xinan Cen, Mangju Wang, Yuhua Sun, Hanyun Ren. POEMS syndrome and Castleman′s disease with renal involvement: a case report and review of the literature[J]. Chinese Journal of Clinicians(Electronic Edition), 2017, 11(17): 2206-2210.

患者,女,46岁,无明显诱因出现"双下肢水肿、皮肤色素沉着,伴畏寒、皮温减低、纳差、腹胀和乏力"。进一步检查显示血清单克隆IgA λ(+)、血管内皮生长因子(VEGF)水平升高;淋巴结肿大、脾肿大;神经病变等。左腹股沟淋巴结活检示Castleman病(浆细胞型)。Castleman病及POEMS综合征诊断明确。予RCP方案(利妥昔单抗、环磷酰胺和地塞米松)联合化疗,上述症状部分缓解,肾功能维持稳定水平。本文对Castleman病引起POEMS综合征,以及Castleman病导致肾功能不全的原因进行文献复习。

A 46-year-old female presented to our hospital with edema of the lower limbs and skin hyperpigmentation. She also complained of chills, low skin temperature, abdominal swelling, poor appetite, and fatigue. Serum protein electrophoresis revealed M protein, and immunofixation assay showed positivity for monoclonal IgA λ. Her VEGF level was elevated. She also had lymphadenopathy, splenomegaly, and neuropathy. Left groin lymph node biopsy suggested Castleman′s disease (plasma cell variant). Thus, a diagnosis of Castleman′s disease and POEMS syndrome was confirmed. She was treated with the RCP regimen (rituximab, cyclophosphamide, and dexamethasone), and her symptoms were partially alleviated. We also reviewed the relevant literature to discuss this rare condition.

图3 左侧腹股沟淋巴结活检(HE染色,A:×40;B:×100)
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