bloodjourГематология и трансфузиологияRussian journal of hematology and transfusiology0234-57302411-3042ООО Издательский дом «Практика»10.35754/0234-5730-2023-68-1-70-79bloodjour-433Research ArticleОБЗОРЫREVIEWSГены иммуноглобулинов и стереотипные антигенные рецепторы при хроническом лимфолейкозе и других лимфопролиферативных заболеванияхImmunoglobulin genes and stereotyped antigenic receptors in chronic lymphocytic leukemia and other lymphoproliferative diseaseshttps://orcid.org/0000-0002-6253-3334БидерманБ. В.BidermanB. V.

Белла Вениаминовна Бидерман, кандидат биологических наук, старший научный сотрудник

лаборатория молекулярной гематологии

125167

Москва

Bella V. Biderman, Cand. Sci. (Biol.), Senior Researcher

Department of Molecular Hematology

125167

Moscow

bella_biderman@mail.ruhttps://orcid.org/0000-0001-9463-9187СудариковА. Б.SudarikovA. B.

Андрей Борисович Судариков, доктор биологических наук, заведующий лабораторией

лаборатория молекулярной гематологии

125167

Москва

Andrey B. Sudarikov, Dr. Sci. (Biol.), Head of Department

Department of Molecular Hematology

125167

Moscow

dusha@blood.ruФГБУ «Национальный медицинский исследовательский центр гематологии» Министерства здравоохранения Российской ФедерацииРоссияNational Medical Research Center for HematologyRussian Federation2023130520236817079Copyright © Бидерман Б.В., Судариков А.Б., 20232023Бидерман Б.В., Судариков А.Б.Biderman B.V., Sudarikov A.B.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.htjournal.ru/jour/article/view/433

   Введение. Мутационный статус генов вариабельного региона тяжелой цепи иммуноглобулинов – важнейший прогностический фактор при хроническом лимфолейкозе (ХЛЛ). При ХЛЛ и других лимфопролиферативных заболеваниях наблюдается значительное сужение репертуара генов вариабельного региона тяжелой цепи иммуноглобулинов (immunoglobulin heavy-chain variable region, IGHV).   Цель — обобщение данных о мутационном статусе и особенностях репертуара генов IGHV и их клиническом значении при ХЛЛ и других лимфопролиферативных заболеваниях.   Основные сведения. Последовательность генов IGHV – уникальный маркер опухолевого клона. Больные ХЛЛ с немутированными генами IGHV отличаются крайне неблагоприятным течением заболевания в отличие от больных ХЛЛ с мутациями. У больных с мутациями IGHV достигается хороший ответ на иммунохимиотерапию, при немутированных IGHV требуется назначение новых таргетных препаратов. Изучение репертуара генов IGHV и стереотипных антигенных рецепторов позволяет выявить дополнительные группы больных ХЛЛ с определенными генетическими и клиническими особенностями. При некоторых других лимфопролиферативных заболеваниях также выявляются стереотипные рецепторы, их клиническое значение не изучено. Такие стереотипные рецепторы специфичны для каждого заболевания.

   Introduction. The mutational status of immunoglobulin heavy chain variable region genes (IGHV) is the most important prognostic factor in chronic lymphocytic leukemia (CLL). Furthermore, a significant narrowing of the IGHV gene repertoire is found in CLL and other lymphoproliferative diseases.   Aim — to review the publication data on the IGHV genes repertoire and mutational status in CLL and other lymphoproliferative diseases regarding their clinical significance.   General information. Nucleotide sequence of rearranged IGHV genes is a unique marker of a tumor clone. CLL patients with unmutated IGHV genes have an extremely unfavorable disease outcome in contrast to the patients with mutated IGHV genes. Patients with mutated IGHV genes benefit from conventional immunochemotherapy, while non-mutated IGHV patients require therapy escalation with new targeted drugs. The study of IGHV genes and stereotyped antigen receptors repertoire makes possible to identify additional groups of CLL patients with specific genetic and clinical features. Stereotype receptors are also detected in other lymphoproliferative diseases, but their clinical significance has not yet been defined. However, stereotyped receptors are found to be disease-specific.

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