Langerhans cell histiocytosis in a 28-year-old patient

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Resumo

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.

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Sobre autores

Maria Samsonova

Research Institute of Pulmonology; Loginov Moscow Clinical Scientific and Practical Center

Email: samary@mail.ru
ORCID ID: 0000-0001-8170-1260
Código SPIN: 9525-9085
Scopus Author ID: 7003974325

MD, Dr. Sci. (Medicine), Head of the Department of Pathological Anatomy; Senior Researcher of the Department of Innovative Pathomorphology

Rússia, 28, Orekhovy Blvd., Moscow, 115682; 1, Novogireevskaya St., Moscow, 111123

Andrey Malyavin

Russian University of Medicine

Email: maliavin@mail.ru
ORCID ID: 0000-0002-6128-5914
Código SPIN: 8264-5394
Scopus Author ID: 6701876872

MD, Dr. Sci. (Medicine), Professor of the Department of Phthisiology and Pulmonology; Chief External Expert Pulmonologist of the Central Federal District, General Secretary of RSMSIM

Rússia, 20/1, Delegatskaya St., Moscow, 127473

Andrey Chernyaev

Research Institute of Pulmonology; Academician Avtsyn Research Institute of Human Morphology

Autor responsável pela correspondência
Email: cheral12@gmail.com
ORCID ID: 0000-0003-0973-9250
Código SPIN: 4433-4567
Scopus Author ID: 7004925753

MD, Dr. Sci. (Medicine), Professor, Chief Researcher of the Department of Pathological Anatomy; Leading Researcher

Rússia, 28, Orekhovy Blvd., Moscow, 115682; 3, Tsyurupa St., Moscow, 117418

Tatyana Adasheva

Russian University of Medicine

Email: adashtv@mail.ru
ORCID ID: 0000-0002-3763-8994
Código SPIN: 6923-0509
Scopus Author ID: 57497595200

MD, Dr. Sci. (Medicine), Professor of the Department of Therapy and Preventive Medicine

Rússia, 20/1, Delegatskaya St., Moscow, 127473

Pavel Asadulin

Russian University of Medicine

Email: asadulin.pavel@yandex.ru
ORCID ID: 0000-0001-5236-1770
Código SPIN: 8196-6454
Scopus Author ID: 57279740100

MD, Head of the Department of Therapy of the Surgical Hospital of University Clinic of the Semashko Scientific and Educational Institute

Rússia, 20/1, Delegatskaya St., Moscow, 127473

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2. Fig. 1. Multispiral computed tomography of the observed patient: A - multiple thin-walled cavities in the lungs of different sizes; B - multiple thin-walled cavities in the lungs, emphysema in the apex of the right lung

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3. Fig. 2. Granuloma consisting of lymphocytes, Langerhans cells, pigmented macrophages and lymphocytes (haematoxylin and eosin staining, ×200)

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4. Fig. 3. Cluster of Langerhans cells expressing langerin (immunohistochemical reaction, ×200)

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