Smirnov A V, Afanasyev B V, Poddubnaya I V, Dobronravov V A, Khrabrova M S, Zakharova E V, Nikitin E A, Lysenko Kozlovskaya L V, Bobkova I N, Rameev V V, Batyushin M M, Moiseev I S, Darskaya E I, Pirogova O V, Mendeleeva L P, Biryukova L S
Pavlov First Saint Petersburg State Medical University.
Russian Medical Academy of Continuous Professional Education.
Ter Arkh. 2020 Sep 1;92(7):10-22. doi: 10.26442/00403660.2020.07.000659.
Monoclonal gammopathy of renal significance (MGRS) is a new nosology in modern nephrology and oncohematology. MGRS is defined as kidney injury due to nephrotoxic monoclonal immunoglobulin produced by the B-cell line clone which does not reach the hematological criteria for specific treatment initiation. Monoclonal proteins pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: 1) limited knowledge about the MGRS among hematologists and nephrologists; 2) lack of necessary diagnostic resources in most health-care facilities; 3) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. Consensus document comprises the opinion of experts leading nephrologists and hematologists of Russian Federation on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy, 1516 of March 2019, Pavlov First Saint Petersburg State Medical University. The present Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses.
具有肾脏意义的单克隆丙种球蛋白病（MGRS）是现代肾脏病学和肿瘤血液学中的一种新的疾病分类。MGRS被定义为由B细胞系克隆产生的肾毒性单克隆免疫球蛋白导致的肾损伤，该克隆未达到启动特异性治疗的血液学标准。单克隆蛋白对肾实质的病理作用导致肾功能不可逆转地下降，直至终末期肾病，这与血液学家和肾脏病学家的国际共识一致，确定了MGRS患者进行克隆特异性治疗的迫切必要性，尽管缺乏启动治疗的血液学指征。俄罗斯联邦MGRS的主要挑战是，由于以下原因，绝大多数患者无法及时获得诊断和适当治疗：1）血液学家和肾脏病学家对MGRS的了解有限；2）大多数医疗机构缺乏必要的诊断资源；3）缺乏针对这种病理实体治疗的批准的临床建议和医疗经济标准。共识文件包含了俄罗斯联邦主要肾脏病学家和血液学家对MGRS问题的意见，包括疾病分类、诊断方法和克隆特异性治疗基本原理方面的不一致。共识文件基于2019年3月15 - 16日在圣彼得堡第一巴甫洛夫国立医科大学浆细胞发育异常和淋巴增殖性疾病：现代治疗方法研讨会框架内举行的俄罗斯主要肾脏病学家和血液学家会议期间达成的结论和协议。本共识旨在确定解决俄罗斯联邦MGRS问题的主要实际步骤，这些步骤总结在最终条款中。
