Morphologic manifestations of combined light-chain deposition disease and light-chain cast nephropathy
Morphologic manifestations of combined light-chain deposition disease and light-chain cast nephropathy
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DOI：
10.1080/01913120701376139
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发表时间：
2007-01-01
影响因子：
1
通讯作者：
Walker, Patrick D.
中科院分区：
文献类型：
--
作者：
Gokden, Neriman;Cetin, Neslihan;Walker, Patrick D.
肾脏轻链沉积病(LCDD)合并轻链铸型肾病(LCCN)的病理形态资料较少。本文报告23例LCDD和LCCN的形态特征。他们用光镜(LIGH)、免疫荧光(IF)和电子显微镜(EM)对23例肾活检进行了回顾性评估。骨髓瘤21例，单克隆性伽玛病1例，未发现疾病1例。结节状肾小球硬化，这是提示LCDD的光镜下病变，仅在23例中发现3例。肾小球不明显者16例(69%)。所有活检组织均可见LCCN的诊断性管型。23例(100%)呈线性轻链(LC)免疫反应，其中13例为GBM+TBM，7例仅为TBM，1例为GBM+间质，1例为GBM、TBM和系膜。15例(65%)可见颗粒状电子致密沉积，其中5例为基底膜，5例为基底膜，4例为基底膜和基底膜，1例为系膜。15例(65%)有IF和EM可探测到的沉积物，8例(37%)有IF可探测到的沉积物。LCCN在所有患者的LM表现中占主导地位。光镜下可见肾小球轻微或无改变。本研究显示在LCDD和LCCN合并病例中LCDD缺乏特征性的LM表现，并强调了在没有IF和EM的情况下很难做出明确的诊断。
There are few data on morphology of light-chain deposition disease (LCDD) of kidney with coexistent light-chain cast nephropathy (LCCN). Here, the authors report the morphology in 23 cases of LCDD and LCCN. They retrospectively evaluated 23 renal biopsies with light ( LM), immunofluorescence ( IF), and electron microscopy ( EM). Twenty-one patients had myeloma, 1 had a monoclonal gammopathy, and in 1 no illness was found. Nodular glomerulosclerosis, the LM lesion suggestive of LCDD, was noted in only 3 of 23 cases. Glomeruli were unremarkable in 16 (69%) cases. The diagnostic casts of LCCN were seen in all biopsies. Linear light chain ( LC) immunoreactivity was observed in 23 (100%) cases ( 18 kappa, 5 lambda); GBM+TBM in 13, TBM only in 7, GBM only in 1, TBM and interstitium in 1, GBM, TBM and mesangium in 1. Casts were positive with same LC in all cases ( 100%). Fifteen cases (65%) showed granular electron-dense deposits; GBM only in 5, TBM only in 5, GBM and TBM in 4, mesangium in 1. In 8 patients without EM deposits, the diagnosis of LCDD was rendered by IF. Fifteen ( 65%) had deposits detectable by IF and EM, 8 (37%) had deposits with IF only. LCCN dominated the LM findings in all patients. There were minimal or no glomerular changes by LM. This study shows the lack of characteristic LM findings of LCDD in combined cases of LCDD and LCCN and emphasizes the difficulty for-definitive diagnosis-without IF and EM.