Terapevticheskii arkhivTerapevticheskii arkhivТерапевтический архив0040-36602309-5342LLC Obyedinennaya Redaktsiya3229710.17116/terarkh201789785-92Editorial articleПередовая статьяShort CommunicationDiffuse large B-cell lymphoma with concomitant c-MYC and BCL6 gene rearrangements with primary skin involvement: A case report and a review of literatureДиффузная В-крупноклеточная лимфома с сочетанной реаранжировкой генов c-MYC и BCL6 с первичным поражением кожи: собственное наблюдение и обзор литературыGabeevaN GГабееваН ГKorolevaD AКоролеваД АBelyaevaA VБеляеваА ВChernovaN GЧерноваН ГKuzminaL AКузьминаЛ АSudarikovA BСудариковА БObukhovaT NОбуховаТ НKovriginaA MКовригинаА МZvonkovE EЗвонковЕ ЕSavchenkoV GСавченкоВ ГФГБУ «Гематологический научный центр» Минздрава России15072017897VOL 89, NO7 ()ТОМ 89, №7 (2017)859210042020Copyright ©; 2017, Consilium MedicumCopyright ©; 2017, ООО "Консилиум Медикум"2017Consilium MedicumООО "Консилиум Медикум"https://creativecommons.org/licenses/by-nc-sa/4.0https://ter-arkhiv.ru/0040-3660/article/view/32297

Double-hit lymphoma (DHL) is a rare aggressive B-cell lymphoma with concomitant c-MYC, BCL2 or BCL6 gene rearrangements, which is characterized by the high frequency of extranodal lesions and by resistance to chemotherapy. The median survival does not exceed 18 months in patients with this disease. The majority of DHL is represented by с-MYC/BCL2 cases. The combination of c-MYC/BCL6 occurs rarely (5—8%). The paper describes a case of DHL with concomitant c-MYC and BCL6 gene rearrangements, which mimics diffuse large B-cell lymphoma, leg-type.

Аннотация «Double-hit» лимфома (DHL) — редкая агрессивная В-клеточная лимфатическая опухоль с сочетанной перестройкой генов с-MYC, BCL2 или BCL6, характеризующаяся высокой частотой экстранодальных поражений и резистентностью к химиотерапии. Медиана продолжительности жизни при данном заболевании не превышает 18 мес. Большинство DHL представлено случаями с-MYC/BCL2. Сочетание с-MYC/BCL6 встречается редко (5—8%). Представлен случай DHL-лимфомы с сочетанной реаранжировкой генов c-MYC и BCL6, протекающей под маской ДВККЛ leg-type.

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