scholarly journals Rare Lung Diseases III: Pulmonary Langerhans’ Cell Histiocytosis

2010 ◽  
Vol 17 (3) ◽  
pp. e55-e62 ◽  
Author(s):  
Stephen C Juvet ◽  
David Hwang ◽  
Gregory P Downey
Keyword(s):  
Cell Biology ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Treatment Options ◽  
Langerhans Cell ◽  
Major Theme ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Current Series ◽  
Pulmonary Manifestations ◽  
Rare Lung Diseases

Pulmonary Langerhans’ cell histiocytosis (PLCH) is an unusual cystic lung disease that is also characterized by extrapulmonary manifestations. The current review discusses the presenting features and relevant diagnostic testing and treatment options for PLCH in the context of a clinical case. While the focus of the present article is adult PLCH and its pulmonary manifestations, it is important for clinicians to distinguish the adult and pediatric forms of the disease, as well as to be alert for possible extrapulmonary complications. A major theme of the current series of articles on rare lung diseases has been the translation of insights gained from fundamental research to the clinic. Accordingly, the understanding of dendritic cell biology in this disease has led to important advances in the care of patients with PLCH.

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

scholarly journals A-CYST-ANCE ON LUNG DISEASES: A CASE OF PULMONARY LANGERHANS CELL HISTIOCYTOSIS

CHEST Journal ◽  
2020 ◽  
Vol 158 (4) ◽  
pp. A1139
Author(s):  
Byung Soo Yoo ◽  
Francis Pham ◽  
Sukhpreet Kaur ◽  
Marina Johnston
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis

scholarly journals Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective

2016 ◽  
Vol 140 (3) ◽  
pp. 230-240 ◽  
Author(s):  
Anja C. Roden ◽  
Eunhee S. Yi
Keyword(s):  
Pulmonary Hypertension ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Additional Treatment ◽  
Langerhans Cell ◽  
Braf V600e ◽  
First Line ◽  
Clonal Proliferation ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Appropriate Treatment

Context Pulmonary Langerhans cell histiocytosis (PLCH) is a rare histiocytic disorder that almost exclusively affects the lungs of smokers. PLCH is characterized by bronchiolocentric nodules and/or cysts in an upper and mid lung distribution with sparing of the costophrenic angles. The diagnosis can be challenging and often requires transbronchial biopsy or surgical lung biopsy. Pulmonary hypertension is a relatively common and sometimes severe complication of PLCH. The pathogenesis of PLCH is still debated. Recently, BRAF V600E mutation and BRAF expression have been identified in some patients with PLCH, suggesting that at least a subset of PLCH has a clonal proliferation. While smoking cessation is the first-line treatment of PLCH, some patients might require additional treatment and eventually transplant. Given that the lesional cells of PLCH express BRAF in some patients, MAPKinase pathway–targeted treatment might be useful for therapy-resistant patients. Objective —To present the more recently recognized clinical and pathologic aspects of PLCH, including pulmonary hypertension in PLCH, pathogenesis, and treatment, as well as the basic diagnostic approach to PLCH. Data Sources Authors' own research, and search of literature database (PubMed) and UpToDate. Conclusions —Despite the recent progress, more studies are needed to elucidate the biology of PLCH for identification of prognostic factors and appropriate treatment options, especially for therapy-refractory PLCH cases.

scholarly journals Smoking and Other Interstitial Lung Diseases

2011 ◽  
Vol 2011 ◽  
pp. 1-7
Author(s):  
Carpio Carlos ◽  
Gómez-Carrera Luis ◽  
Álvarez-Sala Rodolfo
Keyword(s):  
Computed Tomography ◽  
Smoking Cessation ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Langerhans Cell ◽  
Lung Disorder ◽  
High Resolution Computed Tomography ◽  
Pulmonary Langerhans Cell Histiocytosis

Cigarette smoking has been implicated in the development of some uncommon respiratory interstitial diseases. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung diseases are characterized by a diffuse alveolar and peribronchiolar filling with macrophages, respectively. Pulmonary Langerhans' cell histiocytosis is a rare interstitial lung disorder characterized by the proliferation of Langerhans' cell forming interstitial infiltrates and nodules that could progress to cavitary nodules. The treatment of these disorders involves smoking cessation and sometimes the use of steroids. High-resolution computed tomography is essential for the characterization of these smoking-related interstitial lung diseases, but frequently it is necessary to create a workgroup composed by pulmonologists, pathologists, and radiologists to diagnosis and treat patients affected with these pathologies.

scholarly journals Pulmonary histiocytosis

2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 101-105
Author(s):  
Branislava Milenkovic ◽  
Andrija Bogdanovic ◽  
Tijana Cvok ◽  
Javorka Mitic ◽  
Jelena Stojsic ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Bone Lesions ◽  
High Resolution Computed Tomography ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Pulmonary Histiocytosis

Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, and bone marrow. Pulmonary histiocytosis. Adult pulmonary Langerhans cell histiocytosis is a rare disorder of unknown etiology. It occurs predominantly in male smokers, with an incidence peak between 20 and 40 years of age. High-resolution computed tomography of the chest can show nodules, cavitated nodules, and thickand thin-walled cysts. The definite diagnosis of pulmonary Langerhans cell histiocytosis requires identification of Langerhans? cell granulomas infiltrating and destroying distal bronchioles, which is usually achieved by lung biopsy at a site selected by chest high-resolution computed tomography. Treatment. Treatment options for adults have never been clarified by a clinical trial. The published literature provides minimal data on the comparative efficacy of various treatment options which include surgery/curettage, steroids, radiation, and various chemotherapy regimens. The improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans cell histiocytosis should help in the development of specific therapeutic strategies and effective treatment.

Radiologic manifestation of pulmonary Langerhans' cell histiocytosis

1993 ◽  
Vol 29 (5) ◽  
pp. 973
Author(s):  
Jong Sung Kim ◽  
Duk Ja Bang ◽  
Hyun Chul Rhim ◽  
Seok Chol Jeon ◽  
Seung Ro Lee ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Pulmonary Langerhans Cell Histiocytosis
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