Langerhans cell histiocytosis in a 28-year-old patient

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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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作者简介

Maria Samsonova

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

Email: samary@mail.ru
ORCID iD: 0000-0001-8170-1260
SPIN 代码: 9525-9085
Scopus 作者 ID: 7003974325

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

俄罗斯联邦, 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
SPIN 代码: 8264-5394
Scopus 作者 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

俄罗斯联邦, 20/1, Delegatskaya St., Moscow, 127473

Andrey Chernyaev

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

编辑信件的主要联系方式.
Email: cheral12@gmail.com
ORCID iD: 0000-0003-0973-9250
SPIN 代码: 4433-4567
Scopus 作者 ID: 7004925753

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

俄罗斯联邦, 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
SPIN 代码: 6923-0509
Scopus 作者 ID: 57497595200

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

俄罗斯联邦, 20/1, Delegatskaya St., Moscow, 127473

Pavel Asadulin

Russian University of Medicine

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

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

俄罗斯联邦, 20/1, Delegatskaya St., Moscow, 127473

参考

  1. Goyal G, Young JR, Koster MJ, Tobin WO, Vassallo R, Ryu JH et al.; Mayo Clinic Histiocytosis Working Group. The Mayo Clinic Histiocytosis Working Group consensus statement for the diagnosis and evaluation of adult patients with histiocytic neoplasms: Erdheim – Chester disease, Langerhans cell histiocytosis, and Rosai – Dorfman disease. Mayo Clin Proc. 2019;94(10):2054–71. PMID: 31472931. https://doi.org/10.1016/j.mayocp.2019.02.023
  2. Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans’-cell histiocytosis in adults. New Eng J Med. 2002;346(7):484–90. PMID: 11844849. https://doi.org/10.1056/NEJMoa012087
  3. Götz G, Fichter J. Langerhans’-cell histiocytosis in 58 adults. Eur J Med Res. 2004;9(11):510–14. PMID: 15649860.
  4. Mason RH, Foley NM, Branley HM, Adamali HI, Hetzel M, Maher TM, Suntharalingam J. Pulmonary Langerhans cell histiocytosis (PLCH): A new UK register. Thorax. 2014;69(8):766–67. PMID: 24482091. https://doi.org/10.1136/thoraxjnl-2013-204313
  5. Schönfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration. 1993;60(1):38–44. PMID: 8469818. https://doi.org/10.1159/000196171
  6. Brown NA, Elenitoba-Johnson KSJ. Clinical implications of oncogenic mutations in pulmonary Langerhans cell histiocytosis. Curr Opin Pulm Med. 2018;24(3):281–86. PMID: 29470255. https://doi.org/10.1097/MCP.0000000000000470
  7. Allen CE, Li L, Peters TL, Leung H-CE, Yu A, Man T-K et al. Cell-specific gene expression in Langerhans cell histiocytosis lesions reveals a distinct profile compared with epidermal Langerhans cells. J Immunol. 2010;184(8):4557–67. PMID: 20220088. PMCID: PMC3142675. https://doi.org/10.4049/jimmunol.0902336
  8. Kilpatrick SE, Wenger DE, Gilchrist GS, Shives TC, Wollan PC, Unni KK. Langerhans’ cell histiocytosis (histiocytosis X) of bone: A clinicopathologic analysis of 263 pediatric and adult cases. Cancer. 1995;76(12):2471–84. PMID: 8625073. https://doi.org/10.1002/1097-0142(19951215)76:12<2471::aid-cncr2820761211>3.0.co;2-z
  9. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: Diagnosis, natural history, management, and outcome. Cancer. 1999;85(10):2278–90. PMID: 10326709. https://doi.org/10.1002/(sici)1097-0142(19990515)85:10<2278::aid-cncr25>3.0.co;2-u
  10. Phillips M, Allen C, Gerson P, McClain K. Comparison of FDGPET scans to conventional radiography and bone scans in management of Langerhans cell histiocytosis. Pediatric Blood Cancer. 2009;52(1):97–101. PMID: 18951435. https://doi.org/10.1002/pbc.21782
  11. Albano D, Bosio G, Giubbini R, Bertagna F. Role of 18F-FDG PET/CT in patients affected by Langerhans cell histiocytosis. Jpn J Radiol. 2017;35(10):574–83. PMID: 28748503. https://doi.org/10.1007/s11604-017-0668-1
  12. Goyal G, Hu M, Young JR, Vassallo R, Ryu JH, Bennani NN et al. Adult Langerhans cell histiocytosis: A contemporary single-institution series of 186 patients. J Clin Oncol. 2019;37(15_suppl):7018. http://dx.doi.org/10.1200/JCO.2019.37.15_suppl.7018
  13. Makras P, Alexandraki KI, Chrousos GP, Grossman AB, Kaltsas GA. Endocrine manifestations in Langerhans cell histiocytosis. Trends Endocrinol Metab. 2007;18(6):252–57. PMID: 17600725. https://doi.org/10.1016/j.tem.2007.06.003
  14. Le Pavec J, Lorillon G, Jaïs X, Tcherakian C, Feuillet S, Dorfmüller P et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: Clinical characteristics and impact of pulmonary arterial hypertension therapies. Chest. 2012;142(5):1150–57. PMID: 22459770. https://doi.org/10.1378/chest.11-2490
  15. King TE Jr. Bronchoscopy in interstitial lung disease. In: Textbook of bronchoscopy. Feinsilver SH, Fein AM, editors. Baltimore, Md.: Williams & Wilkins. 1995: 185. ISBN: 0-683-03107-4.
  16. Roden AC, Hu X, Kip S, Castellar ERP, Rumilla KM, Vrana JA et al. BRAF V600E expression in Langerhans cell histiocytosis: Clinical and immunohistochemical study on 25 pulmonary and 54 extrapulmonary cases. Am J Surg Pathol. 2014;38(4):548–51. PMID: 24625419. https://doi.org/10.1097/PAS.0000000000000129
  17. Goyal G, Lau D, Nagle AM, Vassallo R, Rech KL, Ryu JH et al.; Mayo Clinic Histiocytosis Working Group. Tumor mutational burden and other predictive immunotherapy markers in histiocytic neoplasms. Blood. 2019;133(14):1607–10. PMID: 30696619. PMCID: PMC6911819. https://doi.org/10.1182/blood-2018-12-8939170

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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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