Pulmonary Langerhans cell histiocytosis in an elderly man
https://doi.org/10.18093/0869-0189-2017-27-3-419-422
Abstract
Langerhans cell histiocytosis (LCH) is a heterogeneous group of diseases with typical accumulation of Langerhans cells in different organs and tissues and formation of granulomas with eosinophil infiltration. Pulmonary LCH in adults is an orphan interstitial lung disease. This disease could occur independently on 15% or to be a part of a multisystemic disease in similar proportion of cases. The clinical course of LCH is various and unpredictable and could vary from asymptomatic course to severe progressive pulmonary injury and respiratory failure. The disease could regress spontaneously in a quarter of patients; have the stable course in 50% and progress in 25% of patients. A case of early-stage pulmonary LCH in a 73-year-old smoker was reported in the article. The diagnose was made according to histological and immunohistochemical investigations of VATS biopsy of the lung tissue. Disseminated injury of the lungs without cysts was found that is typical for early stage of LCH and is difficult for detection. Radiological follow-up revealed rapid progression of the disease.
About the Authors
Mariya V. Samsonova
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia;
A.S. Loginov Moscow State Clinical and Research Center, Moscow Healthcare Department
Russian Federation
Doctor of Medicine, Head of Laboratory of Pathology and Immunology, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Senior Researcher, Laboratory of Pathomorphology, A.S.Loginov Moscow State Clinical and Research Center, Moscow Healthcare Department; tel.: (495) 465-53-84
Russian Federation
Doctor of Medicine, Head of Laboratory of Pathology and Immunology, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Senior Researcher, Laboratory of Pathomorphology, A.S.Loginov Moscow State Clinical and Research Center, Moscow Healthcare Department; tel.: (495) 465-53-84
Andrey L. Chernyaev
Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia;
Federal Research Institute of Human Morphology;
N.I. Pirogov Russian State National Research Medical University, Healthcare Ministry of Russia
Russian Federation
Doctor of Medicine, Professor, Head of Division of Pathology, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Leading Researcher, Laboratory of Clinical Morphology, Federal Research Institute of Human Morphology; Professor at Department of Pathology and Clinical Morphology, Therapeutic Faculty, N.I.Pirogov Russian State National Research Medical University, Healthcare Ministry of Russia; tel.: (495) 465-53-84;
Russian Federation
Doctor of Medicine, Professor, Head of Division of Pathology, Federal Pulmonology Research Institute, Federal Medical and Biological Agency of Russia; Leading Researcher, Laboratory of Clinical Morphology, Federal Research Institute of Human Morphology; Professor at Department of Pathology and Clinical Morphology, Therapeutic Faculty, N.I.Pirogov Russian State National Research Medical University, Healthcare Ministry of Russia; tel.: (495) 465-53-84;
Kirill Yu. Mikhaylichenko
S.I. Spasokukotskiy City Clinical Hospital, Moscow Healthcare Department
Russian Federation
radiologist, S.I.Spasokukotskiy City Clinical Hospital, Moscow Healthcare Department; tel.: (968) 822-63-35;
Russian Federation
radiologist, S.I.Spasokukotskiy City Clinical Hospital, Moscow Healthcare Department; tel.: (968) 822-63-35;
Svetlana N. Zhdankina
L.A. Vorokhobov City Clinical Hospital, Moscow Healthcare Department
Russian Federation
pathologist, L.A.Vorokhobov City Clinical Hospital, Moscow Healthcare Department; (926) 158-96-63;
Russian Federation
pathologist, L.A.Vorokhobov City Clinical Hospital, Moscow Healthcare Department; (926) 158-96-63;
References
1. Voytkovskaya K.S. and Chernyaev A.L. Lung histiocytosis from Langerhans cells. Pul'monologiya. 2013; (1): 90–94 (in Russian).
2. Vassallo R., Ryu J., Schroeder D.R. et al. Clinical outcomes of pulmonary Langerhans’ cell histiocytosis in adults. N. Engl. J. Med. 2002; 346 (7): 484–490. DOI: 10.1056/NEJMoa012087.
3. Colby T.V., Lombard C. Histiocytosis X in the lung. Hum. Pathol. 1983; 14 (10): 847–856.
4. Aerni M.R., Aurby M.C., Maers J.L., Vassallo R. Complete remission of nodular pulmonary Langerhans cell histiocytosis lesions induced by 2-chlorodeoxyadenosine in a non-smoker. Respir. Med. 2008; 102 (2): 316–319. DOI: 10.1016/j.rmed.2007.09.002.
5. Degar B.A., Rollins B.J. Langerhans cell histiocytosis: malignancy or inflammatory disorder doing a great job of imitating one? Dis. Model. Mech. 2009; 2 (9-10): 436–439. DOI: 10.1242/dmm.004010.
6. Tazi A. Adult pulmonary Langerhans’ cell histiocytosis. Eur. Respir. J. 2006; 27 (6): 1272–1285. DOI: 10.1183/09031936.06.00024004.
7. Juvet S.C., Hwang D., Downey G.P. Rare lung diseases III: Pulmonary Langerhans’ cell histiocytosis. Can. Respir. J. 2010; 17 (3): e55–62.
Review
For citations:
Samsonova M.V., Chernyaev A.L., Mikhaylichenko K.Yu., Zhdankina S.N. Pulmonary Langerhans cell histiocytosis in an elderly man. PULMONOLOGIYA. 2017;27(3):419-422. (In Russ.) https://doi.org/10.18093/0869-0189-2017-27-3-419-422
Views:
2771
Email this article 