OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press58510.17650/1818-8346-2022-17-4-16-32HEMATOLOGIC MALIGNANCIES: TREATMENTЛЕЧЕНИЕ ГЕМОБЛАСТОЗОВLangerhans cell histiocytosis in adults: literature reviewГистиоцитоз из клеток Лангерганса у взрослых: обзор литературыhttps://orcid.org/0000-0003-2985-0503PotapenkoV. G.ПотапенкоВ. Г.
Russian Federation

3 Prospekt Dinamo, Saint Petersburg 197110

197110 Санкт-Петербург, пр-кт Динамо, 3

potapenko.vsevolod@mail.ruhttps://orcid.org/0000-0002-9191-5091BaykovV. V.БайковВ. В.
Russian Federation

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

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

https://orcid.org/0000-0001-5087-0192ZinchenkoA. V.ЗинченкоА. В.
Russian Federation

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

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

PotikhonovaN. A.ПотихоноваН. А.
Russian Federation

16 2nd Sovetskaya St., Saint Petersburg 191024

191024 Санкт-Петербург, ул. 2-я Советская, 16

City Clinical Hospital No. 31СПб ГБУЗ «Городская клиническая больница № 31»I.P. Pavlov First Saint Petersburg State Medical University, Ministry of Health of RussiaФГБОУ ВО «Первый Санкт-Петербургский государственный медицинский университет им. акад. И.П. Павлова» Минздрава РоссииRussian Research Institute of Hematology and Transfusiology, Federal Medical and Biological AgencyФГБУ «Российский научно-исследовательский институт гематологии и трансфузиологии Федерального медико-биологического агентства»0711202217416320311202203112022https://oncohematology.abvpress.ru/ongm/article/view/585

Langerhans cells histiocytosis is a variant of malignant histiocytosis. The course and symptoms vary. patients with localized forms have a better prognosis, because local therapy is effective. patients with multifocal forms of histiocytosis receive systemic drug therapy, which cures some of the patients. This review provides up-to-date data about typical presentation of the organ involvement, diagnosis, course and therapy of various forms of Langerhans cells histiocytosis.

Гистиоцитоз из клеток лангерганса относится к клональным заболеваниям системы крови. Течение и симптомы варьируют. прогноз у пациентов с локализованными формами лучше, так как эффективна местная терапия. пациенты с многоочаговыми формами гистиоцитоза получают системную терапию, которая позволяет вылечить часть больных. В обзоре приведены современные данные о характере поражения различных органов, диагностике, течении и терапии гистиоцитоза из клеток лангерганса.

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