Wang X Y, Han W X, Chen S Y, Niu D, Wang X Y, Wang C
Department of Pathology, Shanxi Medical University Second Hospital, Taiyuan 030001, China.
Zhonghua Bing Li Xue Za Zhi. 2024 Aug 8;53(8):809-815. doi: 10.3760/cma.j.cn112151-20240223-00115.
To investigate the clinicopathological features of renal leukocyte chemokine type 2 amyloidosis (ALECT2). The prevalence, clinical characteristics, renal histopathological features, and renal outcome of 15 patients with ALECT2 by kidney biopsy were collected in the Department of Kidney Pathology, Shanxi Medical University Second Hospital, Taiyuan, China from January 1993 to December 2023. Immunohistochemistry and mass spectrometry for amyloid proteins were carried out. Fifteen patients with ALECT2 were included in the study, representing 12.93% (15/116) of the renal biopsy-proven amyloidosis cases. There were 5 males and 10 females. The median age at diagnosis was 61 years. All patients had various degrees of proteinuria; 7 patients had nephrotic syndrome; 3 patients had renal insufficiency; 7 patients had microscopic hematuria. Renal biopsy showed that strongly orangophilic amyloid proteins distributed mainly in the renal cortical interstitium, vascular walls, the glomerular mesangium and/or glomerular basement membrane. Eight cases were diagnosed with ALECT2 alone and 7 cases combined with other renal diseases, including 4 cases with membranous nephropathy, 2 cases with IgA nephropathy, and 1 case with subacute tubular interstitial nephropathy. ALECT2 patients with concurrent renal disease showed a higher proteinuria level than those without (3.48 g/24 h versus 4.58 g/24 h). All patients were corroborated by immunohistochemistry to exhibit the specific location of LECT2 in the amyloid fibrils. Mass spectrometry analysis revealed LECT2 polypeptide in 9 patients. Except two patients with worsening renal function, the others showed stable renal function during the mean follow-up period of 12.5 months. ALECT2 is the second common type of renal amyloidosis in our center. The majority of ALECT2 patients show concurrent renal diseases, with a high rate of membranous nephropathy. Amyloid deposits distribute mainly in the cortical interstitium of the kidney, the glomerular mesangium and vascular walls. Mass spectrometry is the most sensitive and specific method for detecting LECT2 amyloidosis.
探讨肾白细胞趋化因子2型淀粉样变性（ALECT2）的临床病理特征。收集了1993年1月至2023年12月在中国山西省太原市山西医科大学第二医院肾脏病理科经肾活检确诊的15例ALECT2患者的患病率、临床特征、肾脏组织病理学特征及肾脏转归情况。对淀粉样蛋白进行了免疫组织化学和质谱分析。15例ALECT2患者纳入本研究，占经肾活检证实的淀粉样变性病例的12.93%（15/116）。其中男性5例，女性10例。诊断时的中位年龄为61岁。所有患者均有不同程度的蛋白尿；7例患者患有肾病综合征；3例患者有肾功能不全；7例患者有镜下血尿。肾活检显示，强嗜橙红色的淀粉样蛋白主要分布于肾皮质间质、血管壁、肾小球系膜和/或肾小球基底膜。8例单独诊断为ALECT2，7例合并其他肾脏疾病，其中4例为膜性肾病，2例为IgA肾病，1例为亚急性肾小管间质性肾病。合并肾脏疾病的ALECT2患者蛋白尿水平高于未合并者（3.48 g/24 h对4.58 g/24 h）。所有患者经免疫组织化学证实LECT2在淀粉样纤维中有特异性定位。质谱分析在9例患者中检测到LECT2多肽。除2例肾功能恶化患者外，其他患者在平均12.5个月的随访期内肾功能稳定。ALECT2是我们中心第二常见的肾淀粉样变性类型。大多数ALECT2患者合并肾脏疾病，膜性肾病发生率高。淀粉样沉积物主要分布于肾皮质间质、肾小球系膜和血管壁。质谱分析是检测LECT2淀粉样变性最敏感和特异的方法。
