OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press52010.17650/1818-8346-2022-17-1-65-74RARE AND COMPLEX CLINICAL SITUATIONS: DIAGNOSTIC DIFFICULTIE AND TREATMENT CHOICEРЕДКИЕ И СЛОЖНЫЕ КЛИНИЧЕСКИЕ СИТУАЦИИ: ТРУДНОСТИ ДИАГНОСТИКИ И ВЫБОР ТАКТИКИ ЛЕЧЕНИЯMolecular genetic features of cutaneous T-cell lymphomas development on example of mycosis fungoides and Sezary syndromeМолекулярно-генетические особенности развития Т-клеточных лимфом кожи на примере грибовидного микоза и синдрома Сезариhttps://orcid.org/0000-0002-8980-4851KhadzhievaM. B.ХаджиеваМ. Б.
Russian Federation

Maryam B. Khadzhieva

1 Samory Mashela St., Moscow 117198,

Build. 2, 25 Petrovka St., Moscow 107031

Марьям Борисовна Хаджиева

117198 Москва, ул. Саморы Машела, 1,

107031 Москва, ул. Петровка, 25, стр. 2

mkhadzhieva@fnkcrr.ruZakharovaE. S.ЗахароваЕ. С.
Russian Federation

1 Samory Mashela St., Moscow 117198

117198 Москва, ул. Саморы Машела, 1

KalininaE. V.КалининаЕ. В.
Russian Federation

1 Samory Mashela St., Moscow 117198

117198 Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0003-3664-2876AbramovD. S.АбрамовД. С.
Russian Federation

1 Samory Mashela St., Moscow 117198

117198 Москва, ул. Саморы Машела, 1

RumyantsevA. G.РумянцевА. Г.
Russian Federation

1 Samory Mashela St., Moscow 117198

117198 Москва, ул. Саморы Машела, 1

https://orcid.org/0000-0002-2128-0078LarinS. S.ЛаринС. С.
Russian Federation

1 Samory Mashela St., Moscow 117198

117198 Москва, ул. Саморы Машела, 1

Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology, Ministry of Health of RussiaФГБУ «Национальный медицинский исследовательский центр детской гематологии, онкологии и иммунологии им. Дмитрия Рогачева» Минздрава РоссииFederal Research and Clinical Center of Intensive Care Medicine and RehabilitologyФГБНУ «Федеральный научно-клинический центр реаниматологии и реабилитологии»2901202217165742901202229012022https://oncohematology.abvpress.ru/ongm/article/view/520

Cutaneous T-cell lymphomas are a heterogeneous group of T-cell lymphoproliferative diseases affecting the skin. Mycosis fungoides and Sezary syndrome are the most studied variants of them. The literature review includes the latest published data on the pathological processes development in mycosis fungoides and Sezary syndrome and the diagnosis of these diseases. The genomic instability features in cutaneous T-cell lymphomas are described, the existing hypotheses of the origin of these diseases are considered based on the results of T-cell receptor repertoire studying.

Т-клеточные лимфомы кожи представляют собой гетерогенную группу Т-клеточных лимфопролиферативных заболеваний, поражающих кожу. Грибовидный микоз и синдром Сезари – наиболее изученные варианты Т-клеточных лимфом кожи. Обзор литературы включает последние опубликованные данные по развитию патологических процессов при грибовидном микозе и синдроме Сезари и диагностике этих заболеваний. Описаны особенности геномной нестабильности при Т-клеточных лимфомах кожи, рассмотрены существующие гипотезы происхождения данных заболеваний по результатам изучения репертуара Т-клеточного рецептора.

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