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学科主题: 临床医学
题名:
Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction
作者: Yao, Ying; Wang, Su-Xia1; Zhang, You-Kang; Wang, Yan; Liu, Li; Liu, Gang
关键词: Fanconi syndrome ; immunoglobulin light chains ; multiple myeloma
刊名: INTERNAL MEDICINE
发表日期: 2014
DOI: 10.2169/internalmedicine.53.0836
卷: 53, 期:2, 页:121-124
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Medicine, General & Internal
研究领域[WOS]: General & Internal Medicine
英文摘要:

Light chain proximal tubulopathy is a rarely reported entity associated with plasma cell dyscrasia that classically manifests as acquired Fanconi syndrome and is characterized by the presence of kappa-restricted crystals in the proximal tubular cytoplasm. We herein present a case of multiple myeloma with Fanconi syndrome and acute kidney injury due to light chain proximal tubulopathy with light chain cast nephropathy. Prominent phagolysosomes and numerous irregularly shaped inclusions with a fibrillary matrix in the cytoplasm of the proximal tubules were identified on electron microscopy. A monotypic light chain of the lambda type was detected in the distal tubular casts, proximal tubular cytoplasmic lysosomes and fibrillary inclusions on immunofluorescence and immune electron microscopy. This case underscores the importance of conducting careful ultrastructural investigations and immunocytologic examinations of light chains for detecting and diagnosing light chain proximal tubulopathy.

语种: 英语
WOS记录号: WOS:000330216400008
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/56681
Appears in Collections:北京大学第一临床医学院_肾脏内科_期刊论文

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作者单位: 1.Peking Univ, Dept Med, Div Renal, Hosp 1, Beijing, Peoples R China
2.Peking Univ, Inst Nephrol, Beijing, Peoples R China
3.Minist Hlth China, Key Lab Renal Dis, Beijing, Peoples R China

Recommended Citation:
Yao, Ying,Wang, Su-Xia,Zhang, You-Kang,et al. Acquired Fanconi Syndrome with Proximal Tubular Cytoplasmic Fibrillary Inclusions of lambda Light Chain Restriction[J]. INTERNAL MEDICINE,2014,53(2):121-124.
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