Pulmonary Langerhans Cell Histiocytosis.

doi:10.1055/s-0039-1700996

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Article

Pulmonary Langerhans Cell Histiocytosis.

Seminars in respiratory and critical care medicine - 1 Apr 2020

Shaw Brian, Borchers Michael, Zander Dani, Gupta Nishant

Abstract excerpt

Pulmonary 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...

Topics

Clinical Trials as Topic
Disease Progression
Histiocytosis, Langerhans-Cell
Humans
Hypertension, Pulmonary
Lung Diseases
Mitogen-Activated Protein Kinases
Mutation
Prognosis
Proto-Oncogene Proteins B-raf
Respiratory Insufficiency
Smoking
Smoking Cessation

Source

Open in PubMed

Identifiers

PMID: 32279297
DOI: 10.1055/s-0039-1700996

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