Abstract
Pulmonary Langerhans cell histiocytosis (PLCH) is a histiocytic disorder caused by abnormal proliferation of myeloid-derived dendritic cells. Other than the presence of the namesake Langerhans cells, the histopathologic findings in this disorder overlap with many inflammatory and fibrotic conditions that can affect lung tissue. Nodular lung disease with fibrosis and remodeling of alveolar parenchyma are findings common to both PLCH and the organizing pneumonia pattern of injury. Furthermore, organizing pneumonia can be seen in PLCH. This chapter aims to explore this diagnostic challenge and highlight the most useful features to avoid a missed diagnosis of PLCH when it is associated with fibrosis.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Demartino E, Go RS, Vassallo R. Langerhans cell histiocytosis and other histiocytic diseases of the lung. Clin Chest Med. 2016;37(3):421–30.
Pierry C, Caumont C, Blanchard E, Brochet C, Doumes G, Gros A, et al. Assessment of BRAF mutation in pulmonary Langerhans cell histiocytosis in tissue biopsies and bronchoalveolar lavages by droplet digital polymerase chain reaction. Virchows Arch. 2018;472(2):247–58.
Pfeifer K, Mian A, Adebowale A, Alomari A, Kalra V, Krejci E, et al. Radiographic and pathologic manifestations of uncommon and rare pulmonary lesions. Can Assoc Radiol J. 2016;67(2):179–89.
Roden AC, Yi ES. Pulmonary langerhans cell histiocytosis: an update from the pathologists’ perspective. Arch Pathol Lab Med. 2016;140(3):230–40.
Ling CH, Ji C, Raymond DP, Bourne PA, Xu HD. Uncommon features of pulmonary Langerhans’ cell histiocytosis: analysis of 11 cases and a review of the literature. Chin Med J. 2010;123(4):498–501.
Valladeau J, Ravel O, Dezutter-dambuyant C, Moore K, Kleijmeer M, Liu Y, et al. Langerin, a novel C-type lectin specific to Langerhans cells, is an endocytic receptor that induces the formation of Birbeck granules. Immunity. 2000;12(1):71–81.
Lee JW, Lee KS, Lee HY, Chung MP, Yi CA, Kim TS, et al. Cryptogenic organizing pneumonia: serial high-resolution CT findings in 22 patients. AJR Am J Roentgenol. 2010;195(4):916–22.
Larsen BT, Colby TV. Update for pathologists on idiopathic interstitial pneumonias. Arch Pathol Lab Med. 2012;136(10):1234–41.
Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002;19(5):794–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2022 Springer Nature Switzerland AG
About this chapter
Cite this chapter
Long, T.H., Xu, H. (2022). Pulmonary Langerhans Cell Histiocytosis with Fibrosis Versus Organizing Pneumonia. In: Xu, H., Ricciotti, R.W., Mantilla, J.G. (eds) Practical Lung Pathology. Practical Anatomic Pathology. Springer, Cham. https://doi.org/10.1007/978-3-031-14402-8_43
Download citation
DOI: https://doi.org/10.1007/978-3-031-14402-8_43
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-031-14401-1
Online ISBN: 978-3-031-14402-8
eBook Packages: MedicineMedicine (R0)