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Pulmonary Langerhans Cell Histiocytosis^*

Emerging Concepts in Pathobiology, Radiology, and Clinical Evolution of Disease

^* From the Division of Respiratory, Critical Care, and Occupational Medicine (Drs. Sundar and Cahill), Department of Medicine, University of Utah Medical Center, Salt Lake City; and Department of Radiology (Drs. Gosselin and Chung), University of Utah Medical Center, Salt Lake City, UT.

Correspondence to: Krishna M. Sundar, MD, Division of Respiratory, Critical Care, and Occupational Medicine, University of Utah Medical Center, 26 North 1900 East, Salt Lake City, UT 84132-4701; e-mail: krishna.sundar{at}hsc.utah.edu

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.

Key Words: disease progression • eosinophilic granuloma • histiocytosis • histiocytosis X • Langerhans cell • Langerhans cells

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