Light chain deposition disease after renal transplantation.
Light chain deposition disease after renal transplantation.
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肾移植后轻链沉积病。
DOI：
10.1053/j.ajkd.2008.02.370
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
Y. Yamaguchi
中科院分区：
文献类型：
--
作者：
S. Taneda;K. Honda;S. Horita;I. Koyama;S. Teraoka;H. Oda;Y. Yamaguchi
S轻链沉积病是以肾等多器官轻链沉积或免疫球蛋白沉积为特征的疾病。本病常与多发性骨髓瘤或其他淋巴浆增生性疾病有关，尽管相当数量的LCDD患者没有骨髓异常的证据。LCDD在肾移植后经常被报告为EURR，不可避免地会因移植失败而发生。多发性骨髓瘤也被认为是移植肾轻链沉积的主要原因，并表现出较高的复发率。LCDD的最大特点是类似于糖尿病肾小球硬化的结节状肾小球病变。系膜结节由细胞外基质蛋白与单克隆性轻链沉淀物混合而成。肾小球基底膜(GBM)、小管基底膜(TBM)和血管壁因环下轻链沉积而不同程度地增厚。临床上，各种蛋白尿和肾功能不全是常见的表现，病情进展可导致肾功能迅速恶化。我们报告了一例肾移植后4年多的LCDD进展病例，并对系列活检标本进行了回顾性分析，发现移植肾中轻链沉积逐渐进展。
K s ight chain deposition disease (LCDD) is characterized by the deposition of or immuoglobulin light chains in many organs, includng the kidney. The disease frequently is ssociated with multiple myeloma or other lymhoplasmacytic proliferative disorders, although significant number of patients with LCDD how no evidence of bone marrow abnormaliies. LCDD frequently has been reported to ecur after renal transplantation, inevitably folowed by graft failure. Multiple myeloma also as been considered a major cause of light chain eposition in renal allografts and to show a high ate of recurrence. The most characteristic feature of LCDD is odular glomerulopathy resembling diabetic gloerulosclerosis. The mesangial nodules are comosed of extracellular matrix proteins admixed ith the monoclonal light chain deposits. Glomerlar basement membranes (GBMs), tubular baseent membranes (TBMs), and vessel walls are ariably thickened as a consequence of subendohelial light chain deposition. Clinically, various egrees of proteinuria and renal insufficiency are ommon manifestations, and rapid deterioration n kidney function can occur as a consequence of isease progression. We report a case of LCDD progression more han 4 years after renal transplantation, with a etrospective analysis of serial biopsy specimens hat showed gradual progression of light chain eposition in the renal allograft.