open access

Vol 85, No 5 (2017)
REVIEWS
Published online: 2017-10-30
Submitted: 2017-07-12
Accepted: 2017-09-08
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Pulmonary Langerhans' cell histiocytosis in adults

Elżbieta Radzikowska
DOI: 10.5603/ARM.a2017.0046
·
Adv Respir Med 2017;85(5):277-289.

open access

Vol 85, No 5 (2017)
REVIEWS
Published online: 2017-10-30
Submitted: 2017-07-12
Accepted: 2017-09-08

Abstract

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.

Abstract

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.

Get Citation

Keywords

pulmonary Langerhans cell histiocytosis, pneumothorax, caldribine, BRAF

About this article
Title

Pulmonary Langerhans' cell histiocytosis in adults

Journal

Advances in Respiratory Medicine

Issue

Vol 85, No 5 (2017)

Pages

277-289

Published online

2017-10-30

DOI

10.5603/ARM.a2017.0046

Bibliographic record

Adv Respir Med 2017;85(5):277-289.

Keywords

pulmonary Langerhans cell histiocytosis
pneumothorax
caldribine
BRAF

Authors

Elżbieta Radzikowska

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