Моноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапии
Моноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапии
Смирнов А.В., Афанасьев Б.В., Поддубная И.В. и др. Моноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапии. Терапевтический архив. 2020; 92 (7): 10–22. DOI: 10.26442/00403660.2020.07.000659
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Smirnov A.V., Afanasyev B.V., Poddubnaya I.V., et al. Monoclonal gammopathy of renal significance: consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment Therapeutic Archive. 2020; 92 (7): 10–22. DOI: 10.26442/00403660.2020.07.000659
Моноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапии
Смирнов А.В., Афанасьев Б.В., Поддубная И.В. и др. Моноклональная гаммапатия ренального значения: консенсус гематологов и нефрологов России по введению нозологии, диагностике и обоснованности клон-ориентированной терапии. Терапевтический архив. 2020; 92 (7): 10–22. DOI: 10.26442/00403660.2020.07.000659
________________________________________________
Smirnov A.V., Afanasyev B.V., Poddubnaya I.V., et al. Monoclonal gammopathy of renal significance: consensus of hematologists and nephrologists of Russia on the establishment of nosology, diagnostic approach and rationale for clone specific treatment Therapeutic Archive. 2020; 92 (7): 10–22. DOI: 10.26442/00403660.2020.07.000659
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 РФ 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 г. в рамках симпозиума «Плазмоклеточные дискразии и лимфопролиферативные заболевания: новые подходы к терапии», проведенного в ФГБОУ ВО «Первый СПбГМУ им. акад. И.П. Павлова». Настоящий консенсус гематологов и нефрологов призван наметить основные практические пути решения проблемы МГРЗ в РФ, 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: 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”, 15–16 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.
Keywords: monoclonal gammopathy of renal significance, monoclonal gammopathy of undetermined significance, onconephrology, kidney injury, clone specific treatment, paraprotein, kidney biopsy, plasma cell dyscrasias, light chains.
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1. Leung N, Bridoux F, Hutchison CA, et al. Monoclonal gammopathy of renal significance: when MGUS is no longer undetermined or insignificant. Blood. 2012;120(22):4292-5. doi: 10.1182/blood-2012-07-445304
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3. Kozlovskaya LV, Rameev VV, Kogarko IN, et al. Renal lesions associated with monoclonal gammopathies of undetermined significance: clinical forms, mechanisms of development, approaches to treatment. Klin. meditsina. 2016;94(12):892-901 (In Russ.) doi: 10.18821/0023-2149-2016-94-12-892-901
4. Khrabrova MS, Dobronravov VA, Smirnov AV. Kidney disease associated with monoclonal gammopathies: single-center study. Nephrology. 2018;22(6):38-46 (In Russ.) doi: 10.24884/1561-6274-2018-22-6-38-46
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Авторы
А.В. Смирнов1, Б.В. Афанасьев 1, И.В. Поддубная2, В.А. Добронравов1, М.С. Храброва1, Е.В. Захарова2–4, Е.А. Никитин2,4, Л.В. Лысенко (Козловская)5, И.Н. Бобкова5, В.В. Рамеев5, М.М. Батюшин6, И.С. Моисеев1, Е.И. Дарская1, О.В. Пирогова1, Л.П. Менделеева7, Л.С. Бирюкова2,7 – от имени специалистов-нефрологов, специалистов-гематологов Российской Федерации и профессиональных сообществ – участников консенсуса*
1 ФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава России, Санкт-Петербург, Россия;
2 ФГБОУ ДПО «Российская медицинская академия непрерывного профессионального образования» Минздрава России, Москва, Россия;
3 ФГБОУ ВО «Московский государственный медико-стоматологический университет им. А.И. Евдокимова» Минздрава России, Москва, Россия;
4 ГБУЗ «Городская клиническая больница им. С.П. Боткина» Департамента здравоохранения г. Москвы, Москва, Россия;
5 ФГАОУ ВО «Первый Московский государственный медицинский университет им. И.М. Сеченова» Минздрава России (Сеченовский Университет), Москва, Россия;
6 ФГБОУ ВО «Ростовский государственный медицинский университет» Минздрава России, Ростов-на-Дону, Россия;
7 ФГБУ «Национальный медицинский исследовательский центр гематологии» Минздрава России, Москва, Россия
________________________________________________
A.V. Smirnov1, B.V. Afanasyev 1, I.V. Poddubnaya2, V.A. Dobronravov1, M.S. Khrabrova1, E.V. Zakharova2–4, E.A. Nikitin2,4, L.V. Lysenko (Kozlovskaya)5, I.N. Bobkova5, V.V. Rameev5, M.M. Batyushin6, I.S. Moiseev1, E.I. Darskaya1, O.V. Pirogova1,
L.P. Mendeleeva7, L.S. Biryukova2,7 – on behalf of Consensus Participants-nephrologists and hematologists of Russian Federation and professional societies
1 Pavlov First Saint Petersburg State Medical University, Saint Petersburg, Russia;
2 Russian Medical Academy of Continuous Professional Education, Moscow, Russia;
3 Yevdokimov Moscow State University of Medicine and Dentistry, Moscow, Russia;
4 Botkin City Clinical Hospital, Moscow, Russia;
5 Sechenov First Moscow State Medical University (Sechenov University), Moscow, Russia;
6 Rostov State Medical University, Rostov-on-Don, Russia;
7 National Research Center for Hematology, Moscow, Russia