Pulmonary Langerhans cell histiocytosis: A comprehensive analysis of 40 patients and literature review

Elia, Davide; Torre, Olga; Cassandro, Roberto; Caminati, Antonella

doi:10.1016/j.ejim.2015.04.001

Cited by 81 publications

(88 citation statements)

References 43 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…So far, we still know little about the clinical features of PLCH despite several studies have been reported (8,17,18). We hope our study will add more data to PLCH, therefore, facilitate the diagnosis and management.…”

Section: Discussionmentioning

confidence: 99%

“…The pathogenesis of PLCH is uncertain, previous studies found that PLCH had high incidence in smokers (1,(6)(7)(8)(9)(10)(11)(12)(13), particularly in young adult smokers (14), but precise epidemiological data was not available. The relative frequency in men and women is equal (8), which probably because of the increased prevalence of smoking among women in recent years (15). Among adults with PLCH, the long term survival is shorter than that of general population, and their health is substantially affected (14).…”

Section: Original Articlementioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

Li³

et al. 2016

J. Thorac. Dis.

View full text Add to dashboard Cite

Section: Discussionmentioning

confidence: 99%

Section: Original Articlementioning

confidence: 99%

Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

Li³

et al. 2016

J. Thorac. Dis.

View full text Add to dashboard Cite

“…Pulmonary lesions in the course of LCH may take isolated forms that anticipate even for many years the occurrence of systemic changes, or from the very beginning, the lungs may be one www.journals.viamedica.pl of several affected organs. The isolated pulmonary form of LCH is observed in approximately 50% of patients with PLCH [3,[7][8][9][10][11][12].…”

Section: Definitionmentioning

confidence: 99%

“…According to Japanese data, the prevalence of PLCH in women amounts to 0.07/100,000 and 0.27/100 000 in men. Due to the nature of the disorder, it is assessed that the prevalence of PLCH is underestimated [3,[7][8][9][10][11][12][13].…”

Section: Epidemiologymentioning

confidence: 99%

Pulmonary Langerhans' Cell Histiocytosis in Adults

Radzikowska¹

2017

Advances in Respiratory Medicine

View full text Add to dashboard Cite

Pulmonary Langerhans' cell histiocytosis (PLCH) is a rare disorder of unknown cause characterised by the infiltration of the lungs and other organs by the bone marrow derived Langerhans' cells, which carry mutations of BRAF gene and/or NRAS, KRAS and MAP2K1 genes. It occurs predominantly in young smokers, without gender predominance. The disease is characterised by formation of eosinophilic granulomas with the presence of Langerhans' cells infiltrating and destroying distal airways. High-resolution computed tomography of the chest (HRCT) plays an outstanding role in PLCH diagnosis. The typical radiological picture of PLCH is the presence of small intralobular nodules, often forming 'tree in bud' lesions, cavitated nodules, thin-and thick-walled cystic lesions frequently confluent. Definite diagnosis requires the finding of characteristic lesions in histological examination and demonstration of antigen CD1a or CD207 presenting cells in immunohistochemistry. Smoking cessation is the most important recommendation for PLCH patients. There are no evidence based data regarding systemic steroid therapy. The treatment of progressive PLCH is based on cladribine or cytarabine as salvage therapy. The prognosis is good, and over 85% of patients survive 10 years.

show abstract

“…Smoking cessation is essential in all patients and they may need to be enrolled in individualised programmes. It is the only required intervention in a significant proportion of patients [86]. Combined inhaled corticosteroid and long-acting β 2 -agonist therapy may provide benefit to patients with wheezing and obstructive lung disease [13].…”

Section: Treatmentmentioning

confidence: 99%

How I manage pulmonary Langerhans cell histiocytosis

Lorillon

Tazi

2017

Eur Respir Rev

View full text Add to dashboard Cite

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare sporadic cystic lung disease of unknown aetiology that is characterised by the infiltration and destruction of the wall of distal bronchioles by CD1a + Langerhans-like cells. In adults, PLCH is frequently isolated and affects young smokers of both sexes. Recent multicentre studies have led to the more standardised management of patients in clinical practice. Smoking cessation is essential and is occasionally the only suitable intervention. Serial lung function testing is important because a significant proportion of patients may experience an early decline in forced expiratory volume in 1 s and develop airflow obstruction. Cladribine was reported to dramatically improve progressive PLCH in some patients. Its efficacy and tolerance are currently being evaluated. Patients who complain of unexplained dyspnoea with decreased diffusing capacity of the lung for carbon monoxide should be screened for pulmonary hypertension by Doppler echocardiography, which must be confirmed by right heart catheterisation. Lung transplantation is a therapeutic option for patients with advanced PLCH.The identification of the BRAF V600E mutation in approximately half of Langerhans cell histiocytosis lesions, including PLCH, and other mutations of the mitogen-activated protein kinase (MAPK) pathway in a subset of lesions has led to targeted treatments (BRAF and MEK (MAPK kinase) inhibitors). These treatments need to be rigorously evaluated because of their potentially severe side-effects.

show abstract

Pulmonary Langerhans cell histiocytosis: A comprehensive analysis of 40 patients and literature review

Cited by 81 publications

References 43 publications

Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

Pulmonary Langerhans cell histiocytosis: analysis of 14 patients and literature review

Pulmonary Langerhans' Cell Histiocytosis in Adults

How I manage pulmonary Langerhans cell histiocytosis

Contact Info

Product

Resources

About