Castleman病伴以肾病综合征为表现的肾淀粉样变性1例并文献复习  

Castleman disease with renal amyloidosis presenting as nephrotic syndrome:a case report and literature review

作　　者：曹钦婷 白洁菲[1] 刘辉[1] 冯茹[1] CAO Qinting;BAI Jiefei;LIU Hui;FENG Ru(Department of Hematopathology,Bejing Hospital,National Center of Gerontology,Institute of Geriatric Medicine,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing.100730,China)

机构地区：[1]北京医院血液科,国家老年医学中心,中国医学科学院老年医学研究院,北京100730

出　　处：《临床血液学杂志》2023年第3期206-209,共4页Journal of Clinical Hematology

基　　金：北京市自然科学基金面上项目(No:7222158)。

摘　　要：为了探讨Castleman病(CD)伴肾病综合征的肾病理特点及肾AA型淀粉样变与其他肾病理对CD伴肾病综合征预后的影响,回顾性分析1例CD伴肾病综合征且肾活检为AA型淀粉样变患者的临床资料,查阅国内外文献,复习CD伴肾病综合征的肾病理分型以及肾淀粉样变分型与血液疾病关系。结果发现CD伴肾病综合征患者病理以AA型淀粉样变最为常见,多发性骨髓瘤伴肾病综合征肾病理以AL型最多见,AA型淀粉样变较其他肾病理类型预后差。CD引起肾损害少见且隐匿,有些以大量蛋白尿为首发表现,应引起临床医生警惕,AA型淀粉样变预后差,CD合并肾损害的患者应尽早完善肾活检明确诊断。To investigate the renal pathological characteristics of Castleman’s disease(CD)with nephrotic syndrome and the effect of renal AA amyloidosis and other renal pathology on the prognosis of CD with nephrotic syndrome.It retrospectively analyzed clinical data of a patient who had CD with nephrotic syndrome and whose renal biopsy was determined as AA amyloidosis.By reading domestic and foreign literatures,we reviewed the renal pathological classification of CD with nephrotic syndrome and the relationship between renal amyloidosis and blood diseases.AA amyloidosis was the most common pathology in patients with CD with nephrotic syndrome and AL amyloidosis was the most common renal pathology in patients with multiple myeloma with nephrotic syndrome.Furthermore,the prognosis of AA amyloidosis was worse than that of other renal pathological types.CD induced renal damage is rare and insidious and some firstly manifested as a large amount of proteinuria,which should arouse the vigilance of clinicians.The prognosis of AA amyloidosis is poor.Patients with CD complicated with renal damage should complete renal biopsy as soon as possible to confirm the diagnosis.

关 键 词：CASTLEMAN病 肾病综合征 AA型淀粉样变 肾活检 低白蛋白血症 

分 类 号：R557[医药卫生—血液循环系统疾病]