Langerhans cell histiocytosis in a 28-year-old patient
Abstract. Langerhans cell histiocytosis (LCH) is a rare disease. In 50–60% of cases, only lungs are affected. The age of patients with this disease varies from 20 to 40 years. The article presents a clinical case of a 28-year-old patient with a smoking history of 36.5 packs/years, who developed chest pain and discomfort, shortness of breath, heaviness in the back, and a dry cough before admission to the hospital. Computed tomography of chest organs described nodules up to 2 cm in diameter and cystic thin-walled cavities in the upper and middle lobes of the lungs. Over two years, the patient had three spontaneous pneumothoraces. Computed tomography scan of the lungs revealed congenital familial bullous pulmonary disease, serum alpha-1 antitrypsin level was 1.67 g/l. Videothoracoscopic lung biopsy histologically let to reveale macrophage and giant cell pleurisy. A repeat examination of the same biopsy revealed LCH with immunohistochemical confirmation. The patient was diagnosed with LCH three years after the first episode of spontaneous pneumothorax.Samsonova M.V., Malyavin A.G., Chernyaev A.L., Adasheva T.V., Asadulin P.O.
Keywords
Langerhans cell histiocytosis
immunohistochemistry
computed tomography
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About the Authors
Maria V. Samsonova, MD, Dr. Sci. (Medicine), head of the Department of pathological anatomy, Research Institute of Pulmonology of FMBA of Russia, senior researcher at the Department of innovative pathomorphology, Moscow of A.S. Loginov Moscow Clinical Scientific and Practical Center of the Department of Healthcare of Moscow. Address: 115682, Moscow, 28 Orekhovy Boulevard.E-mail: samary@mail.ru
ORCID: https://orcid.org/0000-0001-8170-1260. Scopus ID: 7003974325. eLibrary SPIN: 9525-9085
Andrey G. Malyavin, MD, Dr. Sci. (Medicine), professor, professor of the Department of phthisiology and pulmonology, Russian University of Medicine of the Ministry of Healthcare of Russia, chief external expert – pulmonologist of the Central Federal District, general secretary of RSMSIM. Address: 127473, Moscow, 20/1 Delegatskaya St.
E-mail: maliavin@mail.ru
ORCID: https://orcid.org/0000-0002-6128-5914. Scopus ID: 6701876872. eLibrary SPIN: 8264-5394
Andrey L. Chernyaev, MD, Dr. Sci. (Medicine), professor, chief researcher at the Department of pathological anatomy, Research Institute of Pulmonology of FMBA of Russia, leading researcher at Academician A.P. Avtsyn Research Institute of Human Morphology of Russian Scientific Center of Surgery named after academician B.V. Petrovsky. Address: 115682, Moscow, 28 Orekhovy Boulevard.
E-mail: cheral12@gmail.com
ORCID: https://orcid.org/0000-0003-0973-9250. Scopus ID: 7004925753. eLibrary SPIN: 4433-4567
Tatyana V. Adasheva, MD, Dr. Sci. (Medicine), professor, professor of the Department of therapy and preventive medicine, Russian University of Medicine of the Ministry of Healthcare of Russia. Address: 127473, Moscow, 20/1 Delegatskaya St.
E-mail: adashtv@mail.ru
ORCID: https://orcid.org/0000-0002-3763-8994. Scopus ID: 57497595200. eLibrary SPIN: 6923-0509
Pavel O. Asadulin, MD, head of the Department of therapy of the Surgical Hospital of University Clinic of N.A. Semashko Scientific and Educational Institute, Russian University of Medicine of the Ministry of Healthcare of Russia. Address: 127473, Moscow, 20/1 Delegatskaya St.
E-mail: asadulin.pavel@yandex.ru
ORCID: https://orcid.org/0000-0001-5236-1770. Scopus ID: 57279740100. eLibrary SPIN: 8196-6454
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