ISSN 1006-298X      CN 32-1425/R

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肾脏病与透析肾移植杂志 ›› 2022, Vol. 31 ›› Issue (2): 195-200.DOI: 10.3969/j.issn.1006-298X.2022.02.020

• • 上一篇    

Castleman病变异型POEMS综合征

  

  • 出版日期:2022-04-28 发布日期:2022-04-21

Castleman disease variant of POEMS syndrome

  • Online:2022-04-28 Published:2022-04-21

摘要: 30岁男性,因“水肿、蛋白尿2月余,腹胀、血清肌酐升高1月余”入院。患者于外院肾活检示“血栓性微血管病样病变”,给予对症治疗,但腹胀、肾功能不全进行性加重。转诊国家肾脏疾病临床医学研究中心发现多发淋巴结肿大、肝脾肿大、多浆膜腔积液、血管内皮生长因子升高,单克隆免疫球蛋白阴性。淋巴结活检符合Castleman病,最终确诊为Castleman病变异型POEMS综合征。


关键词: Castleman病变异型POEMS综合征, Castleman病, POEMS综合征, 多浆膜腔积液

Abstract: A 30yearold man was hospitalized for edema and proteinuria followed by progressive abdominal distension and renal dysfunction.His renal biopsy two months ago at the onset of illness revealed a thrombotic microangiopathy lesion.After admission to our hospital,comprehensive examinations were implemented and uncovered multiple lymphnodes enlargement,hepatosplenomegaly,polyserous effusions,elevated vascular endothelial growth factor (VEGF) level.But neither Mprotein nor clonal plasma cells were detectable.Lymph node biopsy confirmed Castleman Disease.Castleman disease variant POEMS syndrome was the final diagnosis.


Key words: Castleman disease variant POEMS syndromeCastleman disease, POEMS syndrome, polyserous effusion