avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2020-10-2-102-118avk-1022Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИМоноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапииMonoclonal Gammopathy of Renal Significance: Consensus of Hematologists and Nephrologists of Russia on the Establishment of Nosology, Diagnostic Approach and Rationale for Clone Specific TreatmentСмирновА. В.SmirnovA. V.

Научно-исследовательский институт нефрологии, кафедра пропедевтики внутренних болезней с клиникой

Санкт-Петербург

Scientific Research Institute of Nephrology, Department of Propaedeutics of Internal diseases

St-Petersburg

АфанасьевБ. В.AfanasyevB. V.

Научно-исследовательский институт детской онкологии, гематологии и трансплантологии костного мозга им. Р.М. Горбачевой, 

Кафедра гематологии, трансфузиологии, трансплантологии факультета постдипломного образования

Санкт-Петербург

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation,

Department of Hematology, Transfusiology, Transplantology of Postgraduate Education Faculty

St-Petersburg

ПоддубнаяИ. В.PoddubnayaI. V.

Кафедра онкологии

Санкт-Петербург

Oncology Department

Moscow

ДобронравовВ. А.DobronravovV. A.

Научно-исследовательский институт нефрологии, кафедра пропедевтики внутренних болезней с клиникой

Санкт-Петербург

Scientific Research Institute of Nephrology, Department of Propaedeutics of Internal diseases

St-Petersburg

https://orcid.org/0000-0002-8141-4488ХраброваМ. С.KhrabrovaM. S.

Мария Сергеевна Храброва

Кафедра пропедевтики внутренних болезней с клиникой

Санкт-Петербург

Maria S. Khrabrova

Department of Propaedeutics of Internal diseases

St-Petersburg

hrabrovamc@gmail.comЗахароваЕ. В.ZakharovaE. V.

Кафедра нефрологии, Московский государственный медико-стоматологический университет им. А.И. Евдокимова,

Кафедра нефрологии и диализа, Российская медицинская академия непрерывного профессионального образования,

Отделение нефрологии, Городская клиническая больница им. С.П. Боткина

Москва

Nephrology Department, A.I. Yevdokimov Moscow State University of Medicine and Dentistry,

Nephrology and dialysis Department, Russian Medical Academy of Postgraduate Education,

Nephrology Department, City Botkin Memorial Hospital

Moscow

НикитинЕ. А.NikitinE. A.

Кафедра гематологии и трансфузиологии, Российская медицинская академия непрерывного профессионального образования, 

Дневной стационар гематологии, онкологии и химиотерапии городского гематологического центра, Городская клиническая больница им. С.П. Боткина

Москва

Department of Hematology and Transfusiology, Russian Medical Academy of Postgraduate Education,

Outpatient department for hematology, oncology and chemotherapy, City Botkin Memorial Hospital

Moscow

КозловскаяЛ. В.KozlovskayaL. V.

Кафедра внутренних, профессиональных болезней и ревматологии Института клинической медицины

Москва

Department of Internal and occupational diseases and rheumatology

Moscow

БобковаИ. Н.BobkovaI. N.

Кафедра внутренних, профессиональных болезней и ревматологии Института клинической медицины,

Научно-исследовательской отдел Здоровье сберегающих технологий Научно-технологического парка биомедицины

Москва

Department of Internal and occupational diseases and rheumatology,

Biomedical Science and Technology Park

Moscow

РамеевВ. В.RameevV. V.

Кафедра внутренних, профессиональных болезней и ревматологии Института клинической медицины

Москва

Department of Internal and occupational diseases and rheumatology

Moscow

БатюшинМ. М.BatyushinM. M.

Кафедра внутренних болезней № 2

Ростов-на-Дону

Department of Internal diseases № 2

Rostov-on-Don

МоисеевИ. С.MoiseevШю S.

Научно-исследовательский институт детской онкологии, гематологии и трансплантологии костного мозга им. Р.М. Горбачевой,

Кафедра гематологии, трансфузиологии, трансплантологии факультета постдипломного образования

Санкт-Петербург

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation,

Department of Hematology, Transfusiology, Transplantology of Postgraduate Education Faculty

St-Petersburg

ДарскаяЕ. И.DarskayaE. I.

Научно-исследовательский институт детской онкологии, гематологии и трансплантологии костного мозга им. Р.М. Горбачевой

Санкт-Петербург

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation

St-Petersburg

ПироговаО. В.PirogovaO. V.

Научно-исследовательский институт детской онкологии, гематологии и трансплантологии костного мозга им. Р.М. Горбачевой

Санкт-Петербург

Raisa Gorbacheva Memorial Research Institute of Children Oncology, Hematology and Transplantation

St-Petersburg

МенделееваЛ. П.MendeleevaL. P.БирюковаЛ. С.BiryukovaL. S.

Кафедра нефрологии и диализа, Российская медицинская академия непрерывного профессионального образования,

Москва

Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. ПавловаРоссияPavlov First St-Petersburg State Medical UniversityRussian FederationРоссийская медицинская академия непрерывного профессионального образованияРоссияRussian Medical Academy of Postgraduate EducationRussian FederationМосковский государственный медико-стоматологический университет им. А.И. Евдокимова; Российская медицинская академия непрерывного профессионального образования; Городская клиническая больница им. С.П. БоткинаРоссияA.I. Yevdokimov Moscow State University of Medicine and Dentistry; Russian Medical Academy of Postgraduate Education; City Botkin Memorial HospitalRussian FederationРоссийская медицинская академия непрерывного профессионального образования; Городская клиническая больница им. С.П. БоткинаРоссияRussian Medical Academy of Postgraduate Education; City Botkin Memorial HospitalRussian FederationПервый Московский государственный медицинский университет им. И.М. Сеченова (Сеченовский Университет)РоссияI.M. Sechenov First Moscow State Medical University (Sechenov University)Russian FederationРостовский государственный медицинский университетРоссияRostov State Medical UniversityRussian FederationНациональный медицинский исследовательский центр гематологииRussian FederationРоссийская медицинская академия непрерывного профессионального образования; Национальный медицинский исследовательский центр гематологииРоссияNephrology and dialysis Department, Russian Medical Academy of Postgraduate Education; National Research Center of HematologyRussian Federation202031032020102102118Copyright © Смирнов А.В., Афанасьев Б.В., Поддубная И.В., Добронравов В.А., Храброва М.С., Захарова Е.В., Никитин Е.А., Козловская Л.В., Бобкова И.Н., Рамеев В.В., Батюшин М.М., Моисеев И.С., Дарская Е.И., Пирогова О.В., Менделеева Л.П., Бирюкова Л.С., 20202020Смирнов А.В., Афанасьев Б.В., Поддубная И.В., Добронравов В.А., Храброва М.С., Захарова Е.В., Никитин Е.А., Козловская Л.В., Бобкова И.Н., Рамеев В.В., Батюшин М.М., Моисеев И.С., Дарская Е.И., Пирогова О.В., Менделеева Л.П., Бирюкова Л.С.Smirnov A.V., Afanasyev B.V., Poddubnaya I.V., Dobronravov V.A., Khrabrova M.S., Zakharova E.V., Nikitin E.A., Kozlovskaya L.V., Bobkova I.N., Rameev V.V., Batyushin M.M., Moiseev Ш.S., Darskaya E.I., Pirogova O.V., Mendeleeva L.P., Biryukova L.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/1022

Моноклональная гаммапатия ренального значения (МГРЗ) представляет собой новую нозологическую группу в современной нефрологии и онкогематологии. Под МГРЗ понимают поражение почек, обусловленное действием нефротоксичного моноклонального иммуноглобулина, продуцируемого клоном В-клеточной линии, который не достигает критериев, необходимых для начала противоопухолевой терапии по онкогематологическим показаниям. Результатом воздействия моноклонального белка на почечную паренхиму является неуклонное прогрессирование дисфункции почек вплоть до утраты функции органа, что в соответствии с единой позицией международного консенсуса гематологов и нефрологов определенно указывает на необходимость клон-ориентированного лечения МГРЗ, несмотря на отсутствие критериальных онкогематологических показаний. Основной проблемой МГРЗ в Российской Федерации является недоступность для большинства пациентов своевременной диагностики и лечения данной патологии, что обусловлено, во-первых, недостаточной осведомленностью гематологов и нефрологов страны в отношении МГРЗ, во-вторых, отсутствием в большинстве медицинских учреждений необходимых диагностических ресурсов, в-третьих, отсутствием утвержденных рекомендаций и медико-экономических стандартов лечения этой болезни. Текст настоящего Консенсуса заключает в себе мнение специалистов Российской Федерации в отношении нозологической классификации, диагностики и подходов к терапии МГРЗ и основан на итогах проведенного совместного совещания ведущих гематологов и нефрологов страны. Совещание состоялось 15–16 марта 2019 года в рамках симпозиума «Плазмоклеточные дискразии и лимфопролиферативные заболевания: новые подходы к терапии», проведенного в Первом Санкт-Петербургском государственном медицинском университете им. акад. И.П. Павлова. Настоящий Консенсус гематологов и нефрологов призван наметить основные практические пути решения проблемы МГРЗ в Российской Федерации, которые кратко сформулированы в виде заключительных положений.

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 healthcare 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.

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The authors declare that there are no conflicts of interest present.