Pulmonary Langerhans cell histiocytosis: Emerging concepts in pathobiology, radiology, and clinical evolution of disease

Krishna M. Sundar, Marc V. Gosselin, Hannah Chung, Barbara C. Cahill

Research output: Contribution to journalReview article

113 Citations (Scopus)

Abstract

Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon disorder of adult smokers associated with a significant morbidity. Arising from the aberrant accumulation of Langerhans and other immune cells, PLCH tends to cause a relatively isolated pulmonary involvement as compared to other forms of Langerhans cell (LC) and histiocytic disorders. Increased knowledge of cytokine triggers, dendritic cell trafficking, and clonality of LC populations in PLCH have resulted in an improved understanding of the pathobiology of PLCH. High-resolution CT (HRCT) of the chest has led to better appreciation of nodular and cystic radiographic abnormalities characteristic of the disease. Correlation of HRCT abnormalities with lung pathologic changes has led to an improved comprehension of clinical evolution of PLCH. Current clinical predictors for PLCH outcomes remain poor, although long-term follow-up and radiologic monitoring may help to define disease progression. This review discusses advances in PLCH emphasizing the etiopathologic bases of the disease and currently available radiologic modalities for monitoring disease progression.

Original languageEnglish (US)
Pages (from-to)1673-1683
Number of pages11
JournalChest
Volume123
Issue number5
DOIs
StatePublished - May 1 2003

Fingerprint

Langerhans Cell Histiocytosis
Radiology
Lung
Langerhans Cells
Disease Progression
Dendritic Cells
Thorax
Cytokines
Morbidity

Keywords

  • Disease progression
  • Eosinophilic granuloma
  • Histiocytosis
  • Histiocytosis X
  • Langerhans cell
  • Langerhans cells

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine

Cite this

Pulmonary Langerhans cell histiocytosis : Emerging concepts in pathobiology, radiology, and clinical evolution of disease. / Sundar, Krishna M.; Gosselin, Marc V.; Chung, Hannah; Cahill, Barbara C.

In: Chest, Vol. 123, No. 5, 01.05.2003, p. 1673-1683.

Research output: Contribution to journalReview article

Sundar, Krishna M. ; Gosselin, Marc V. ; Chung, Hannah ; Cahill, Barbara C. / Pulmonary Langerhans cell histiocytosis : Emerging concepts in pathobiology, radiology, and clinical evolution of disease. In: Chest. 2003 ; Vol. 123, No. 5. pp. 1673-1683.
@article{36d542d6124e454394a79d29ea61ec5e,
title = "Pulmonary Langerhans cell histiocytosis: Emerging concepts in pathobiology, radiology, and clinical evolution of disease",
abstract = "Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon disorder of adult smokers associated with a significant morbidity. Arising from the aberrant accumulation of Langerhans and other immune cells, PLCH tends to cause a relatively isolated pulmonary involvement as compared to other forms of Langerhans cell (LC) and histiocytic disorders. Increased knowledge of cytokine triggers, dendritic cell trafficking, and clonality of LC populations in PLCH have resulted in an improved understanding of the pathobiology of PLCH. High-resolution CT (HRCT) of the chest has led to better appreciation of nodular and cystic radiographic abnormalities characteristic of the disease. Correlation of HRCT abnormalities with lung pathologic changes has led to an improved comprehension of clinical evolution of PLCH. Current clinical predictors for PLCH outcomes remain poor, although long-term follow-up and radiologic monitoring may help to define disease progression. This review discusses advances in PLCH emphasizing the etiopathologic bases of the disease and currently available radiologic modalities for monitoring disease progression.",
keywords = "Disease progression, Eosinophilic granuloma, Histiocytosis, Histiocytosis X, Langerhans cell, Langerhans cells",
author = "Sundar, {Krishna M.} and Gosselin, {Marc V.} and Hannah Chung and Cahill, {Barbara C.}",
year = "2003",
month = "5",
day = "1",
doi = "10.1378/chest.123.5.1673",
language = "English (US)",
volume = "123",
pages = "1673--1683",
journal = "Chest",
issn = "0012-3692",
publisher = "American College of Chest Physicians",
number = "5",

}

TY - JOUR

T1 - Pulmonary Langerhans cell histiocytosis

T2 - Emerging concepts in pathobiology, radiology, and clinical evolution of disease

AU - Sundar, Krishna M.

AU - Gosselin, Marc V.

AU - Chung, Hannah

AU - Cahill, Barbara C.

PY - 2003/5/1

Y1 - 2003/5/1

N2 - Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon disorder of adult smokers associated with a significant morbidity. Arising from the aberrant accumulation of Langerhans and other immune cells, PLCH tends to cause a relatively isolated pulmonary involvement as compared to other forms of Langerhans cell (LC) and histiocytic disorders. Increased knowledge of cytokine triggers, dendritic cell trafficking, and clonality of LC populations in PLCH have resulted in an improved understanding of the pathobiology of PLCH. High-resolution CT (HRCT) of the chest has led to better appreciation of nodular and cystic radiographic abnormalities characteristic of the disease. Correlation of HRCT abnormalities with lung pathologic changes has led to an improved comprehension of clinical evolution of PLCH. Current clinical predictors for PLCH outcomes remain poor, although long-term follow-up and radiologic monitoring may help to define disease progression. This review discusses advances in PLCH emphasizing the etiopathologic bases of the disease and currently available radiologic modalities for monitoring disease progression.

AB - Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon disorder of adult smokers associated with a significant morbidity. Arising from the aberrant accumulation of Langerhans and other immune cells, PLCH tends to cause a relatively isolated pulmonary involvement as compared to other forms of Langerhans cell (LC) and histiocytic disorders. Increased knowledge of cytokine triggers, dendritic cell trafficking, and clonality of LC populations in PLCH have resulted in an improved understanding of the pathobiology of PLCH. High-resolution CT (HRCT) of the chest has led to better appreciation of nodular and cystic radiographic abnormalities characteristic of the disease. Correlation of HRCT abnormalities with lung pathologic changes has led to an improved comprehension of clinical evolution of PLCH. Current clinical predictors for PLCH outcomes remain poor, although long-term follow-up and radiologic monitoring may help to define disease progression. This review discusses advances in PLCH emphasizing the etiopathologic bases of the disease and currently available radiologic modalities for monitoring disease progression.

KW - Disease progression

KW - Eosinophilic granuloma

KW - Histiocytosis

KW - Histiocytosis X

KW - Langerhans cell

KW - Langerhans cells

UR - http://www.scopus.com/inward/record.url?scp=0038555324&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0038555324&partnerID=8YFLogxK

U2 - 10.1378/chest.123.5.1673

DO - 10.1378/chest.123.5.1673

M3 - Review article

C2 - 12740289

AN - SCOPUS:0038555324

VL - 123

SP - 1673

EP - 1683

JO - Chest

JF - Chest

SN - 0012-3692

IS - 5

ER -