How I manage pulmonary Langerhans cell histiocytosis.

doi:10.1183/16000617.0070-2017

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Article

How I manage pulmonary Langerhans cell histiocytosis.

European respiratory review : an official journal of the European Respiratory Society - 30 Sept 2017

Lorillon Gwenaël, Tazi Abdellatif

Abstract excerpt

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 o...

Topics

Adult
Cladribine
Female
Genetic Predisposition to Disease
Histiocytosis, Langerhans-Cell
Humans
Immunosuppressive Agents
Lung
Lung Diseases
Lung Transplantation
Male
Middle Aged

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Molecular Targeted Therapy

Mutation

Phenotype

Protein Kinase Inhibitors

Proto-Oncogene Proteins B-raf

Respiratory Function Tests

Source

Open in PubMed Open full text

Identifiers

PMID: 28877978
DOI: 10.1183/16000617.0070-2017
PMCID: PMC9489169

Genes

BRAF

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026. Prepared record publication-28877978-2026-08-30.1. Manifest publication-graph-cohort-2026-08-30.1.

Source receipt: PubMed via Helena Literature Corpus, retrieved 30 Aug 2026.

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