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Review
. 2021 Mar 8:7:582581.
doi: 10.3389/fmed.2020.582581. eCollection 2020.

Update on Pulmonary Langerhans Cell Histiocytosis

Elzbieta Radzikowska  1
Affiliations
Review

Update on Pulmonary Langerhans Cell Histiocytosis

Elzbieta Radzikowska. Front Med (Lausanne). .

Abstract

Pulmonary Langerhans cell (LC) histiocytosis (PLCH) has unknown cause and is a rare neoplastic disorder characterized by the infiltration of lungs and various organs by bone marrow-derived Langerhans cells with an accompanying strong inflammatory response. These cells carry somatic mutations of BRAF gene and/or NRAS, KRAS, and MAP2K1 genes, which cause activation of the mitogen-activated protein kinase (MAPK)/extracellular signal-regulated kinase (ERK) signaling pathway. PLCH occurs predominantly in young smokers, without gender predominance. Lungs might be involved as an isolated organ or as part of a multiorgan disease. High-resolution computed chest tomography plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, "tree in bud" opacities, cavitated nodules, and thin- and thick-walled cysts, frequently confluent. Histological examination of the lesion and demonstration of characteristic eosinophilic granulomas with the presence of LCs that display antigen CD1a or CD207 in immunohistochemistry are required for definite diagnosis. Smoking cessation is the most important recommendation for PLCH patients, but treatment of progressive PLCH and multisystem disease is based on chemotherapy. Recently, new targeted therapies have been implemented.

Keywords: BRAF; MAPK; adults; lung; pulmonary Langerhans cell histiocytosis.

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Conflict of interest statement

The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
MEK-mitogen activated protein kinase–extracellular signal-regulated kinase (ERK) signaling cascade of the mitogen-activated protein kinase (MAPK) pathway and phosphoinositide 3-kinase (PI3K)–AKT-serine/threonine protein kinase Akt pathway. Diagram of RAS protein activation.
Figure 2
Figure 2
X-ray film of a pulmonary Langerhans cell histiocytosis (PLCH) patient. Multiple reticular and nodular changes in the upper and middle parts of both lungs, with an increase in lung volume.
Figure 3
Figure 3
High-resolution computed tomography (HRCT) scan of a pulmonary Langerhans cell histiocytosis (PLCH) patient. Multiple intralobular nodules, nodules with cavitations, and small nodular lesions in both lungs.
Figure 4
Figure 4
High-resolution computed tomography (HRCT) scan of a pulmonary Langerhans cell histiocytosis (PLCH) patient. Multiple cystic lesions, frequently confluent with bizarre shapes.
Figure 5
Figure 5
High-resolution computed tomography (HRCT) scan of a pulmonary Langerhans cell histiocytosis (PLCH) patient. Large confluent cystic lesions in both lungs.
Figure 6
Figure 6
Management of pulmonary Langerhans cell histiocytosis (PLCH).
See this image and copyright information in PMC

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