anatomyЖурнал анатомии и гистопатологииJournal of Anatomy and Histopathology2225-7357N.N. Burdenko Voronezh State Medical University10.18499/2225-7357-2023-12-4-89-97anatomy-1842Research ArticleОБЗОРНЫЕ СТАТЬИREVIEW ARTICLESПатоморфологическая характеристика лимфангиолейомиоматоза легкихPathomorphological Characteristic of Pulmonary Lymphangioleiomyomatosishttps://orcid.org/0000-0002-8633-8496ПоловниковИ. В.PolovnikovI. V.

Половников Илья Вячеславович – врач-пульмонолог

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

Il'ya V. Polovnikov – pulmonologist

ul. L'va Tolstogo, 6-8, St. Petersburg, 197022

policrats@mail.ruhttps://orcid.org/0000-0001-8888-4135ЮкинаГ. Ю.YukinaG. Yu.

Юкина Галина Юрьева – зав. лабораторией патоморфологии научно-клнического центра патоморфологии

Санкт-Петербург

Galina Yu. Yukina – head laboratory of pathomorphology of the scientific and clinical center of pathomorphology

St-Petersburg

pipson@inbox.ruhttps://orcid.org/0000-0001-5521-7248СухоруковаЕ. Г.SukhorukovaE. G.

Сухорукова Елена Геннадьевна – старший научный сотрудник лаборатории патоморфологии научноклнического центра патоморфологии

Санкт-Петербург

Elena G. Sukhorukova – senior researcher of laboratory of pathomorphology, scientific and clinical center of pathomorphology

St-Petersburg

len48@inbox.ruПервый Санкт-Петербургский государственный медицинский университет им. акад. И.П. ПавловаРоссияAcademician I.P. Pavlov First St. Petersburg State Medical UniversityRussian Federation2023190120241248997Copyright © Половников И.В., Юкина Г.Ю., Сухорукова Е.Г., 20242024Половников И.В., Юкина Г.Ю., Сухорукова Е.Г.Polovnikov I.V., Yukina G.Y., Sukhorukova E.G.This work is licensed under a Creative Commons Attribution 4.0 License.https://anatomy.elpub.ru/jour/article/view/1842

Лимфангиолейомиоматоз (ЛАМ) – полисистемное заболевание, в основе которого лежит образование склонных к перерождению в кисты гранулем в легких, а также органах брюшной полости и малого таза. Поражение легких при ЛАМ сопровождается нарушением функции дыхания и может приводить к летальному исходу. ВОЗ относит это заболевание к группе мезенхимальных опухолей и считает его метастазирующим новообразованием низкой степени злокачественности. ЛАМ является орфанным заболеванием, и ранее считалось, что оно поражает только лиц женского пола, однако на настоящий момент описаны случаи заболевания у мужчин, в том числе младше 18 лет. Открытым остается вопрос этиологии ЛАМ и факторов, провоцирующих это заболевание. Цель обзора – сбор и систематизация данных о патоморфологической характеристике изменений, происходящих в тканях легкого при ЛАМ, оценка полноты и достаточности имеющихся данных. Материал и методы. Проанализировано 912 статей и монографий (включая дубли) из баз PubMed, eLibrary, Cyberleninka за пятидесятилетний период с 1973 г. по август 2023 г. с акцентом на более актуальные публикации и статьи в высокорейтинговых научных журналах, включающие информацию по теме обзора. Отобрано 55 оригинальных источников. Результаты. В статье представлены сводные данные о макроскопической и микроскопической картине поражения легких при ЛАМ, данные о морфологической и антигенной характеристике клеток, составляющих основу гранулем и их окружения, результаты экспериментов, демонстрирующих динамику патологических изменений при ЛАМ. Заключение. Несмотря на относительно большое количество данных о морфологической характеристике изменений при ЛАМ, достаточных для диагностики, открытым к настоящему времени остается вопрос о происхождение клеток ЛАМ, ответ на который лежит, в том числе, в плоскости системного патоморфологического анализа.

Lymphangioleiomyomatosis (LAM) is a polysystemic disease based on the formation of granulomas prone to degenerate into cysts in the lungs, abdominal and pelvic organs. Lung involvement in LAM is accompanied by respiratory dysfunction and can be fatal. WHO classifies this disease into a group of mesenchymal tumors and considers it as a low-grade destructive metastasizing neoplasm. LAM is a rare disease and earlier it was only detected in women, but nowadays cases of pulmonary LAM in men (include teenagers) have been described. The etiology of LAM and the inducing factors are still unknown. Objective – to collect and systematize data on pathomorphological characteristic of changes in lung tissue in LAM to evaluate the completeness and sufficiency of the available data. Material and methods. There were analyzed 912 articles and monographs (including duplicates) from PubMed, eLibrary, and Cyberleninka databases over a fifty-year period from 1973 to August of 2023, with an emphasis on more relevant publications and articles in highly rated scientific journals that include information on the review topic. Eventually 55 original sources were selected. Results. The article presents summary data on macroscopic and microscopic picture of pulmonary LAM, data on morphological and antigenic characterization of cells forming the basis of granulomas and their surroundings, the results of experiments demonstrating dynamics of pathological changes in LAM. Conclusion. Despite the relatively large amount of data about morphological characteristics that is enough for diagnosis, he main issue is still open: what is the origin of LAM cells? A systematic pathomorphology analyze can help us to find an answer for this question.

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The authors declare that there are no conflicts of interest present.