Almanac of Clinical MedicineAlmanac of Clinical MedicineАльманах клинической медицины2072-05052587-9294Moscow Regional Research and Clinical Institute (MONIKI)158710.18786/2072-0505-2022-50-057REVIEW ARTICLEОБЗОРResearch ArticleLangerhans cell histiocytosis as a clonal disease of mononuclear phagocyte systemГистиоцитоз из клеток Лангерганса как клональное заболевание системы моноцитов/макрофаговhttps://orcid.org/0000-0003-4368-8469KhynkuEvgeniy F.ХынкуЕвгений Федорович
Russian Federation

Junior Research Fellow, Department of Dermatovenereology and Dermato-Oncology

мл. науч. сотр. отделения дерматовенерологии и дерматоонкологии

khynky_ev@mail.ruhttps://orcid.org/0000-0001-8479-1978MonaenkovaMaria K.МонаенковаМария Кирилловна
Russian Federation

Attending Physician, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

врач-ординатор кафедры дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

marymona@yandex.comhttps://orcid.org/0000-0003-3261-6718TamrazovaOlga B.ТамразоваОльга Борисовна
Russian Federation

MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

д-р мед. наук, профессор, профессор кафедры дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

anait_tamrazova@mail.ruhttps://orcid.org/0000-0001-5056-374XTaganovAlexey V.ТагановАлексей Викторович
Russian Federation

MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

д-р мед. наук, профессор, профессор кафедры дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

matis87177@yandex.ruhttps://orcid.org/0000-0001-8212-6210GureevaМarina А.ГурееваМарина Александровна
Russian Federation

Assistant Professor, Head of Teaching Department, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

ассистент, заведующая учебной частью кафедры дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

marina.gureeva@mail.ruhttps://orcid.org/0000-0003-4615-7356BagramovaGayane E.БаграмоваГаянэ Эрнстовна
Russian Federation

MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

д-р мед. наук, профессор кафедры дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

khynky_ev@mail.ruhttps://orcid.org/0000-0002-6456-998XMolochkovAnton V.МолочковАнтон Владимирович
Russian Federation

MD, PhD, Professor, Head of Chair of Dermatovenereology and Dermato-Oncology, Postgraduate Training Faculty; Head of Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty

д-р мед. наук, профессор, заведующий кафедрой дерматовенерологии и дерматоонкологии факультета усовершенствования врачей; заведующий кафедрой дерматовенерологии с курсом косметологии факультета непрерывного медицинского образования

antmd@yandex.ruMoscow Regional Research and Clinical Institute (MONIKI)ГБУЗ МО «Московский областной научно-исследовательский клинический институт им. М.Ф. Владимирского»Peoples' Friendship University of RussiaФГАОУ ВО «Российский университет дружбы народов»221220225074284382311202130012023Copyright ©; 2022, Khynku E.F., Monaenkova M.K., Tamrazova O.B., Taganov A.V., Gureeva М.А., Bagramova G.E., Molochkov A.V.Copyright ©; 2022, Хынку Е.Ф., Монаенкова М.К., Тамразова О.Б., Таганов А.В., Гуреева М.А., Баграмова Г.Э., Молочков А.В.2022Khynku E.F., Monaenkova M.K., Tamrazova O.B., Taganov A.V., Gureeva М.А., Bagramova G.E., Molochkov A.V.Хынку Е.Ф., Монаенкова М.К., Тамразова О.Б., Таганов А.В., Гуреева М.А., Баграмова Г.Э., Молочков А.В.https://creativecommons.org/licenses/by-nc/4.0https://almclinmed.ru/jour/article/view/1587

Langerhans cell histiocytosis (LCH) belongs to histiocytic proliferative diseases, which are rare in clinical practice; however they pose significant challenges both for their diagnosis and choice of therapeutic strategies. Histiocytic proliferative diseases are the scope of oncology; nevertheless, at the diagnostic stage the patients are referred to pediatricians or dermatologists. That is why the interdisciplinary interaction of various specialties and common approaches to their classification, diagnosis and treatment are important for the management of patients with histiocytic proliferative disorders.

Accumulation of the studies on the LCH pathophysiology has promoted the development of new diagnostic algorithms and treatment methods. After the fact of MAPK signal pathway activation had been established, the potential target for therapy was identified. Neoplastic nature of LCH has been hypothesized. If confirmed, we can expect actual diagnostic algorithms being elaborated, in particular, the potential to predict the disease depending on the tumor clone mutation type. The unique characteristics of LCH including proliferate clonality (presumable of neoplastic nature), the disease course with spontaneous regression and frequent relapses and tropism to certain tissues (target organs) make up the grounds for further in-depth studies of the disease.

Гистиоцитоз из клеток Лангерганса (ГКЛ) относится к гистиоцитарным пролиферативным заболеваниям, редко встречающимся в клинической практике, однако представляющим значительные трудности как в плане диагностики, так и в плане выбора терапевтических стратегий. Заболевания этой группы входят в сферу деятельности онкологов, но на этапе диагностики пациенты обращаются к педиатрам или дерматологам. В этой связи при ведении пациентов с гистиоцитарными пролиферативными заболеваниями большое значение имеет междисциплинарное взаимодействие и общность подходов врачей разных специальностей к их классификации, диагностике и лечению. Настоящий обзор посвящен пересмотру традиционных представлений об этиопатогенезе ГКЛ.

Накопление данных исследований, посвященных патогенезу ГКЛ, способствовало разработке новых диагностических алгоритмов и методов терапии. После установления факта активации сигнального пути МАРК определилась потенциальная точка приложения таргетной терапии. Предложена гипотеза о неопластической природе ГКЛ. В случае ее подтверждения следует ожидать актуализацию диагностических алгоритмов, в частности появится возможность прогнозировать заболевание в зависимости от типа мутаций опухолевого клона. Уникальные особенности ГКЛ, включая клональность пролиферата (предположительно неопластической природы), течение со спонтанными регрессами и частыми рецидивами и тропизм к определенным тканям (органам-мишеням), формируют предпосылки для дальнейшего углубленного изучения данного заболевания.

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