Monoclonal gammopathy of renal signifi cance: Consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment

Abstract

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 protein’s 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: i) limited knowledge about the MGRS among hematologists and nephrologists; ii) lack of necessary diagnostic resources in most health-care facilities; iii) 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», 15-16 of March 2019, Pavlov First St-Petersburg State Medical University, St-Petersburg, Russia. 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.

Plasma Cell Tumors in Hematological Classifications of 2022: WHO-HAEM5 (WHO, 5th edition) and ICC (International Consensus Classification). A Clinician’s View

“…Эксперты ВОЗ в классификации WHO-HAEM5 включили МГРЗ в раздел плазмоклеточных опухолей [1]. Такое положение представляется чрезвычайно важным, поскольку в силу отсутствия МГРЗ в перечне гематологических злокачественных опухолей эта категория пациентов в нашей стране была исключительно предметом заботы нефрологического сообщества, не имевшего реального доступа и возможности применения современных противоопухолевых препаратов [36].…”

“…Диагностика опухолевой лимфопролиферации требует выполнения иммунофенотипических и молекулярных исследований, направленных на выявление В-клеточного клона. Идентификация М-протеина в крови и моче проводится с использованием методов иммунофиксации и Freelite для определения СЛЦ [36]. Помимо этого требуется обязательное выполнение нефробиопсии с гистологическим, иммуноморфологическим и ультраструктурным исследованиями, позволяющими подтвердить специфическое поражение почек.…”