Almanac of Clinical MedicineAlmanac of Clinical Medicine2072-05052587-9294Moscow Regional Research and Clinical Institute (MONIKI)158710.18786/2072-0505-2022-50-057Research ArticleLangerhans cell histiocytosis as a clonal disease of mononuclear phagocyte systemKhynkuEvgeniy F.<p>Junior Research Fellow, Department of Dermatovenereology and Dermato-Oncology</p>khynky_ev@mail.ruhttps://orcid.org/0000-0003-4368-8469MonaenkovaMaria K.<p>Attending Physician, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty</p>marymona@yandex.comhttps://orcid.org/0000-0001-8479-1978TamrazovaOlga B.<p>MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty</p>anait_tamrazova@mail.ruhttps://orcid.org/0000-0003-3261-6718TaganovAlexey V.<p>MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty</p>matis87177@yandex.ruhttps://orcid.org/0000-0001-5056-374XGureevaМarina А.<p>Assistant Professor, Head of Teaching Department, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty</p>marina.gureeva@mail.ruhttps://orcid.org/0000-0001-8212-6210BagramovaGayane E.<p>MD, PhD, Professor, Chair of Dermatovenereology with a Course of Cosmetology, Continuous Medical Education Faculty</p>khynky_ev@mail.ruhttps://orcid.org/0000-0003-4615-7356MolochkovAnton V.<p>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</p>antmd@yandex.ruhttps://orcid.org/0000-0002-6456-998XMoscow Regional Research and Clinical Institute (MONIKI)Peoples' Friendship University of Russia090220235074284382311202130012023Copyright © 2023, Khynku E.F., Monaenkova M.K., Tamrazova O.B., Taganov A.V., Gureeva М.А., Bagramova G.E., Molochkov A.V.2023<p>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.</p>
<p>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.</p>Langerhans cell histiocytosisMAPK pathwayBRAF-V600E mutationtarget therapy for Langerhans cell histiocytosisгистиоцитоз из клеток ЛангергансаMAPK-путьмутация BRAF-V600Eтаргетная терапия гистиоцитоза из клеток Лангерганса[Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J, Requena-Caballero L, Jordan MB, Abdel-Wahab O, Allen CE, Charlotte F, Diamond EL, Egeler RM, Fischer A, Herrera JG, Henter JI, Janku F, Merad M, Picarsic J, Rodriguez-Galindo C, Rollins BJ, Tazi A, Vassallo R, Weiss LM; Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. 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