An Unusual Case of Lysosomal Paraprotein Accumulation in Glomerular Endothelial Cells
An Unusual Case of Lysosomal Paraprotein Accumulation in Glomerular Endothelial Cells
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肾小球内皮细胞中溶酶体副蛋白积累的异常病例
DOI：
10.1016/j.ekir.2019.09.012
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发表时间：
2020
影响因子：
6
通讯作者：
Oda Hideaki
中科院分区：
文献类型：
--
作者：
Taneda Sekiko;Honda Kazuho;Horita Shigeru;Matsue Kosei;Usui Yoshiaki;Mitobe Michihiro;Ogura Shota;Nitta Kosaku;Oda Hideaki
浆细胞异位症(PCDS)的特征是异常产生大量的IGs，主要是具有肾毒性的游离轻链(LCS)。PCDS患者发生各种肾脏疾病，如管型肾病(CN)、淀粉样变性、LC沉积病(LCDD)和LC近端小管病变。Cast肾病的特征是肾小管内单克隆性LC的管型形成，导致肾功能障碍。LCDD和淀粉样变性是由单克隆性LCS沉积引起的，而LC近端小管病变是由单克隆性LCS直接损伤小管引起的，其特征是近端肾小管细胞中含有单克隆性LCS的溶酶体肿胀或数量增加。近端肾小管上皮细胞1、2中最常见的是含有单克隆性LC的大量增大的溶酶体，1例PCD 3患者肾小球内有巨噬细胞浸润，但在肾小球固有细胞中很少观察到这种溶酶体。我们在此报告一位多发性骨髓瘤(MM)患者，他发展为罕见的肾脏表现。
Plasma cell dyscrasias (PCDs) are characterized by the excessive production of abnormal Igs, mainly free light chains (LCs) with nephrotoxic properties. Patients with PCDs develop a variety of renal diseases such as cast nephropathy (CN), amyloidosis, LC deposition disease (LCDD), and LC proximal tubulopathy. Cast nephopathy is characterized by intratubular cast formation of monoclonal LCs leading to renal dysfunction. LCDD and amyloidosis result from the deposition of monoclonal LCs, and LC proximal tubulopathy is caused by direct tubular damage due to monoclonal LCs and characterized by crystals or by an increased number of swollen lysosomes containing monoclonal LCs in proximal tubular cells. The presence of many enlarged lysosomes containing monoclonal LCs is documented most frequently in proximal tubular cells 1, 2 and was reported in macrophages infiltrating the glomeruli in 1 patient with PCD 3; however, such lysosomes are rarely observed in glomerular intrinsic cells. We herein report the case of a patient with multiple myeloma (MM) who developed a rare renal manifestation.
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DOI：
--
发表时间：
1992
期刊：
影响因子：
--
作者：
S. Eya;T. Takano
通讯作者：
T. Takano
浆细胞恶液质的肾脏表现：从患者床边到研究实验室的评估。
影响因子：
2
作者：
G. Herrera
通讯作者：
G. Herrera
DOI：
10.1053/j.ajkd.2008.02.370
发表时间：
2008
期刊：
American journal of kidney diseases : the official journal of the National Kidney Foundation
影响因子：
--
作者：
S. Taneda;K. Honda;S. Horita;I. Koyama;S. Teraoka;H. Oda;Y. Yamaguchi
通讯作者：
Y. Yamaguchi
影响因子：
4.6
作者：
Vankalakunti M;Bonu R;Shetty S;Siddini V;Babu K;Ballal SH
通讯作者：
Ballal SH
影响因子：
13.2
作者：
Taneda, Sekiko;Honda, Kazuho;Oda, Hideaki
通讯作者：
Oda, Hideaki