OncohematologyOncohematologyОнкогематология1818-83462413-4023Publishing House ABV Press51510.17650/1818-8346-2022-17-1-10-25HEMATOLOGIC MALIGNANCIES: TREATMENTЛЕЧЕНИЕ ГЕМОБЛАСТОЗОВHistiocytic neoplasms: modern aspects of pathogenesis, classification, diagnosis and treatmentГистиоцитарные опухоли: современные аспекты патогенеза, классификации, диагностики и леченияhttps://orcid.org/0000-0002-0050-0721GurievaO. D.ГурьеваО. Д.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478

115478 Москва, Каширское шоссе, 24

https://orcid.org/0000-0002-1469-2365ValievT. T.ВалиевТ. Т.
Russian Federation

Timur T. Valiev

24 Kashirskoe Shosse, Moscow 115478

Тимур Теймуразович Валиев

115478 Москва, Каширское шоссе, 24

timurvaliev@mail.ruhttps://orcid.org/0000-0002-6300-0062PavlovskayaA. I.ПавловскаяА. И.
Russian Federation

24 Kashirskoe Shosse, Moscow 115478

115478 Москва, Каширское шоссе, 24

N.N. Blokhin National Medical Research Center of Oncology, Ministry of Health of RussiaФГБУ «Национальный медицинский исследовательский центр онкологии им. Н.Н. Блохина» Минздрава России2901202217110252801202228012022https://oncohematology.abvpress.ru/ongm/article/view/515

Histiocytic cell tumors present as a difficult group of diseases in terms of differential diagnosis due to their rare frequency, heterogeneous clinical manifestations and ambiguous clinical course. Considering MAPK-signaling pathway mutations role (including BRAFV600E) in the pathogenesis of histiocytic cell tumors the importance of genetic studies in the diagnosis and choice of therapeutic strategy in these diseases increases. In the current issue modern classifications of histiocytic cell tumors, morphological and immunophenotypic features of histiocytic and dendritic cells, and the role of recurrent somatic mutations of the MAPK signaling pathway in the pathological histiocytic proliferation are presented. Particular attention is given to Langerhans cell histiocytosis, Erdheim–Chester disease and juvenile xanthogranuloma. Clinical and diagnostic characteristics of these diseases and therapeutic approaches are presented.

Гистиоцитарные опухоли представляют собой сложную в дифференциально-диагностическом плане группу заболеваний, что обусловлено редкой встречаемостью, пестротой клинических проявлений, неоднозначным клиническим течением. С учетом определения роли мутаций сигнального пути MAPK (в том числе BRAFV600E) в патогенезе гистиоцитарных опухолей возрастает роль генетических исследований в диагностике и выборе тактики терапии при данных заболеваниях. В настоящей статье представлены современные классификации гистиоцитарных опухолей, обозначены морфоиммунологические особенности гистиоцитарных и дендритных клеток, роль рекуррентных соматических мутаций сигнального пути MAPK в патологической гистиоцитарной пролиферации. Особое внимание уделено гистиоцитозу из клеток Лангерганса, болезни Эрдгейма–Честера и ювенильной ксантогранулеме. Приведены клинико-диагностические особенности данных заболеваний, освещены терапевтические подходы.

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