Clinical Medicine Research

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Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report

Received: 21 May 2017    Accepted: 28 June 2017    Published: 26 July 2017
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Abstract

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with a Langerhans cell phenotype, which comprises a wide range of clinical presentations. The reported patient is a 55-year old female with multy-sistem LCH and pulmonary tuberculosis. During diagnostic processing, infiltrates of Langerhans cells (S-100+, CD1a+) in the epidermis, intestinal mucosa and bone marrow, were discovered. Transbronchial needle biopsy of the lung revealed non-specific clusters of macrophages (CD68+, CD1a-). Although CD1a was negative, positive CD68, thoracic MSCT and clinical findings indicated pulmonary LCH. Corticosteroid treatment led to significant clinical improvement. Seven months after treatment with corticosteroids the patient developed a spontaneous left-sided pneumothorax. Thoracic HR (high resolution)-MSCT was highly suggestive for active Mycobacterium tuberculosis (MT) infection together with the positive QUANTIFERON-TB test and typical symptoms. One week after starting standard triple antituberculotic therapy the patient developed hypovolemic shock. Despite all medical efforts, the patient died on the thirteenth day of intensive treatment for multiple organ failure.

DOI 10.11648/j.cmr.20170604.15
Published in Clinical Medicine Research (Volume 6, Issue 4, July 2017)
Page(s) 139-142
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Langerhans-Cell Histiocytosis, Pulmonary Tuberculosis, Multislice Computed Tomography, Corticosteroids, Multiple Organ Failure

References
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[2] Berres M, Merad M, Allen C. Progress in understanding the pathogenesis of Langerhans cell histiocytosis: back to Histiocytosis X?. British Journal of Haematology. 2014; 169(1):3-13.
[3] Özen M, Akçakaya N, Camcioğlu Y, Cokugras H, Oz B. Isolated pulmonary Langerhans-cell histiocytosis mimicking miliary tuberculosis. J Pediatr Inf 2009; 3: 135−7.
[4] Juvet S, Hwang D, Downey G. Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis. Canadian Respiratory Journal. 2010; 17(3):e55-e62.
[5] Dimitropoulos C, Vamvakaris I, Vassos D, Hamalakis G, Gkogkou C, Kokkini A, Ntanos I. Pulmonary Langerhans’ cell histiocytosis or miliary tuberculosis? Case report and literature review. Arch Hell Med 2011; 28(6): 809–13.
[6] Okutan O, Kartaloglu Z, Ilvan A, Deniz O, Silit E, Gorur R. Active pulmonary tuberculosis in a patient with Langerhans cell histiocytosis. Saudi Med J 2006; 27(3):401–2.
[7] Sharma P, Dhingra K, Sural S, Mandal A, Singh T. Langerhans cell histiocytosis masquerading as tuberculosis: A diagnostic dilemma resulting in inappropriate anti-tubercular therapy. Pediatr Blood Cancer. 2009; 53(1):111-3.
[8] Zhang LMoss J. Genetic studies yield clues to the pathogenesis of Langerhans cell histiocytosis. Eur Respir J. 2016; 47(6):1629-31.
[9] Pfeifer K, Mian A, Adebowale A, Alomari A, Kalra V, Krejci E, Shin MS. Radiographic and Pathologic Manifestations of Uncommon and Rare Pulmonary Lesions. Canadian Association of Radiologists Journal. 2016; 67(2):179-89.
[10] Patne S, Dwivedi S, Katiyar R, Gupta V, Gupta A. Langerhans cell histiocytosis diagnosed by FNAC of lymph nodes. J Can Res Ther. 2015; 11(4):1028.
[11] Routy B, Hoang J, Gruber J. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation. Case Reports in Hematology. 2015; 2015: 1-5.
[12] Watanabe R, Tatsumi K, Hashimoto S, Tamakoshi A, Kuriyama T. The Respiratory Failure Research Gr. Clinico-Epidemiological Features of Pulmonary Histiocytosis X. Intern Med. 2001; 40 (10):998-1003.
[13] Suri H, Yi E, Nowakowski G, Vassallo R. Pulmonary langerhans cell histiocytosis. Orphanet Journal of Rare Diseases. 2012; 7 (1):16.
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Author Information
  • Department of Pulmonology, Clinical Hospital Centre Rijeka, Rijeka, Croatia

  • Primary Health Care Office, Health Centre of Primorsko-Goranska County, Rijeka, Croatia

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  • APA Style

    Veljko Flego, Darian Volaric. (2017). Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report. Clinical Medicine Research, 6(4), 139-142. https://doi.org/10.11648/j.cmr.20170604.15

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    ACS Style

    Veljko Flego; Darian Volaric. Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report. Clin. Med. Res. 2017, 6(4), 139-142. doi: 10.11648/j.cmr.20170604.15

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    AMA Style

    Veljko Flego, Darian Volaric. Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report. Clin Med Res. 2017;6(4):139-142. doi: 10.11648/j.cmr.20170604.15

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  • @article{10.11648/j.cmr.20170604.15,
      author = {Veljko Flego and Darian Volaric},
      title = {Multisystem Langerhans Cell Histiocytosis with Fatal Outcome in Adult Patient: A Case Report},
      journal = {Clinical Medicine Research},
      volume = {6},
      number = {4},
      pages = {139-142},
      doi = {10.11648/j.cmr.20170604.15},
      url = {https://doi.org/10.11648/j.cmr.20170604.15},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.cmr.20170604.15},
      abstract = {Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with a Langerhans cell phenotype, which comprises a wide range of clinical presentations. The reported patient is a 55-year old female with multy-sistem LCH and pulmonary tuberculosis. During diagnostic processing, infiltrates of Langerhans cells (S-100+, CD1a+) in the epidermis, intestinal mucosa and bone marrow, were discovered. Transbronchial needle biopsy of the lung revealed non-specific clusters of macrophages (CD68+, CD1a-). Although CD1a was negative, positive CD68, thoracic MSCT and clinical findings indicated pulmonary LCH. Corticosteroid treatment led to significant clinical improvement. Seven months after treatment with corticosteroids the patient developed a spontaneous left-sided pneumothorax. Thoracic HR (high resolution)-MSCT was highly suggestive for active Mycobacterium tuberculosis (MT) infection together with the positive QUANTIFERON-TB test and typical symptoms. One week after starting standard triple antituberculotic therapy the patient developed hypovolemic shock. Despite all medical efforts, the patient died on the thirteenth day of intensive treatment for multiple organ failure.},
     year = {2017}
    }
    

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    AU  - Veljko Flego
    AU  - Darian Volaric
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    AB  - Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal proliferation of cells with a Langerhans cell phenotype, which comprises a wide range of clinical presentations. The reported patient is a 55-year old female with multy-sistem LCH and pulmonary tuberculosis. During diagnostic processing, infiltrates of Langerhans cells (S-100+, CD1a+) in the epidermis, intestinal mucosa and bone marrow, were discovered. Transbronchial needle biopsy of the lung revealed non-specific clusters of macrophages (CD68+, CD1a-). Although CD1a was negative, positive CD68, thoracic MSCT and clinical findings indicated pulmonary LCH. Corticosteroid treatment led to significant clinical improvement. Seven months after treatment with corticosteroids the patient developed a spontaneous left-sided pneumothorax. Thoracic HR (high resolution)-MSCT was highly suggestive for active Mycobacterium tuberculosis (MT) infection together with the positive QUANTIFERON-TB test and typical symptoms. One week after starting standard triple antituberculotic therapy the patient developed hypovolemic shock. Despite all medical efforts, the patient died on the thirteenth day of intensive treatment for multiple organ failure.
    VL  - 6
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