spbmedicalrecordsНовые Санкт-Петербургские врачебные ведомостиNew St. Petersburg Medical Records1609-2201Первый Санкт-Петербургский государственный медицинский университет имени академика И.П. Павлова10.24884/1609-2201-2024-103-3-31-40spbmedicalrecords-30Research ArticleОБЗОРЫ ЛИТЕРАТУРЫLITERATURE REVIEWSЭкстраренальные формы болезни отложения моноклональных иммуноглобулинов: механизмы, клиническая презентация, диагностика, лечение (обзор литературы)Extrarenal monoclonal immunoglobulin deposition disease: mechanisms, clinical presentation, diagnostics and treatment approaches (review)https://orcid.org/0000-0002-8141-4488ХраброваМ. С.KhrabrovaM. S.

Храброва Мария Сергеевна, кандидат медицинских наук, доцент кафедры пропедевтики внутренних болезней с клиникой им. акад. М. Д. Тушинского

197022, Санкт-Петербург, ул. Льва Толстого, д. 6–8

Maria S. Khrabrova, Cand of Sci. (Med.), Associate Professor of the Pavlov University, Department of Propaedeutic of Internal Diseases, Associate Professor of the Department of Propaedeutics of Internal Diseases with clinic named after acad. M. D. Tushinksy

6–8, L’va Tolstogo str., Saint Petersburg, 197022

hrabrovamc@gmail.comПервый Санкт-Петербургский государственный медицинский университет имени академика И. П. ПавловаРоссияPavlov UniversityRussian Federation202427012025033140Copyright © Храброва М.С., 20252025Храброва М.С.Khrabrova M.S.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.docved.ru/jour/article/view/30

При моноклональных гаммапатиях клон В-клеточной линии продуцирует моноклональный иммуноглобулин (МИГ), представляющий собой молекулу или одной легкой цепи κ или λ, или одной тяжелой цепи, или полную молекулу иммуноглобулина. В силу аберрантной природы клона и наличия в его генотипе разнообразных мутаций МИГ может оказывать патологическое воздействие на органы и ткани, в том числе аккумулироваться в виде неорганизованных конго-рот негативных гранулярных депозитов. Такой вариант органотоксичности носит название болезни отложения МИГ. Наиболее частая локализация подобных депозитов МИГ – ренальная паренхима. В то же время, подобно AL-амилоидозу, болезнь отложения МИГ может носить системный характер, изолированно поражать разные органы или протекать в виде локальной формы. В настоящем сообщении суммированы текущие представления о механизмах экстраренальной формы болезни отложения МИГ, ее клинических проявлениях, подходах к диагностике и лечению.

In monoclonal gammopathies the aberrant B-cell clone produces the monoclonal immunoglobulin (MIG) which could present as only one light chain or only one heavy chain or the whole immunoglobulin. Due to somatic mutations in B-cell clone genetic the MIG obtains abnormal features and different types of tissue toxicity. The condition of non-organized granular MIG deposition leading to organ damage and dysfunction is known as monoclonal immunoglobulin deposition disease (MIDD). Most commonly MIDD involves the kidney parenchyma. However, extrarenal MIDD may affect other tissues and present as local or systemic condition. This review summarizes the current knowledge concerning the mechanisms, clinical manifestation, diagnostics and treatment approaches in extrarenal MIDD.

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