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An update to the pathogenesis for monoclonal gammopathy of renal significance.
Annals of Hematology ( IF 3.5 ) Pub Date : 2020-02-26 , DOI: 10.1007/s00277-020-03971-1
Chao Zuo 1, 2 , Yuge Zhu 3 , Gaosi Xu 1
Affiliation  

Monoclonal gammopathy of renal significance (MGRS) is characterized by the nephrotoxic monoclonal immunoglobulin secreted by an otherwise asymptomatic or indolent B cell or plasma cell clone, without hematologic criteria for treatment. These MGRS-associated diseases can involve one or more renal compartments, including glomeruli, tubules, and vessels. Hydrophobic residue replacement, N-glycosylated, increase in isoelectric point in monoclonal immunoglobulin (MIg) causes it to transform from soluble form to tissue deposition, and consequently resulting in glomerular damage. In addition to MIg deposition, complement deposition is also found in C3 glomerulopathy with monoclonal glomerulopathy, which is caused by an abnormality of the alternative pathway and may involve multiple factors including complement component 3 nephritic factor, anti-complement factor auto-antibodies, or MIg which directly cleaves C3. Furthermore, inflammatory factors, growth factors, and virus infection may also participate in the development of the diseases. In this review, for the first time, we discussed current highlights in the mechanism of MGRS-related lesions.

中文翻译:

具有肾脏意义的单克隆性丙种球蛋白病发病机制的更新。

具有肾脏重要意义的单克隆丙种球蛋白病(MGRS)的特征是肾无症状的单克隆免疫球蛋白由无症状或惰性B细胞或浆细胞克隆分泌,没有血液学上的治疗标准。这些与MGRS相关的疾病可能涉及一个或多个肾小室,包括肾小球,肾小管和血管。疏水性残基置换(N-糖基化)增加了单克隆免疫球蛋白(MIg)的等电点,导致其从可溶形式转变为组织沉积,从而导致肾小球损害。除了MIg沉积外,在C3肾小球病伴单克隆肾小球病中也发现补体沉积,这是由替代途径异常引起的,可能涉及多种因素,包括补体成分3肾病因子,抗补体因子自身抗体或直接裂解C3的MIg。此外,炎性因子,生长因子和病毒感染也可能参与疾病的发展。在这篇综述中,我们第一次讨论了与MGRS相关的病变机制的最新进展。
更新日期:2020-02-26
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