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Pulmonary Langerhans cell histiocytosis

Author
Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

Pulmonary Langerhans cell histiocytosis (PLCH), also called eosinophilic granuloma of the lung, pulmonary Langerhans cell granulomatosis, and pulmonary histiocytosis X, is an uncommon interstitial lung disease that primarily affects young adults [1-7]. The true incidence and prevalence are unknown. However, the diagnosis is made in less than 5 percent of lung biopsies and has been seen in less than 2 percent of the patients presenting to the Denver Specialized Center of Research program in Interstitial Lung Disease (a total of approximately 3000 patients over 14 years). No occupational or geographic predisposition has been reported, but nearly all affected individuals have a history of current or prior cigarette smoking. Thus, tobacco smoke is thought to be an etiologic factor.

Pulmonary Langerhans cell histiocytosis will be reviewed here. A general review of Langerhans cell histiocytosis, including the extrapulmonary manifestations of this disorder, is presented separately. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis".)

PATHOLOGY

The Langerhans cell, a differentiated cell of the monocyte-macrophage line, is the pathologic cell type of pulmonary Langerhans cell histiocytosis. It is distinguished by pale staining cytoplasm, a large nucleus and nucleoli, and, on electron microscopy, by classic pentalaminar cytoplasmic inclusions or Birbeck granules (X-bodies (picture 1)). Langerhans cells also demonstrate positive immunohistochemical staining for S100 protein. Another characteristic is the strong presence of CD1 antigen (CD1a) on the cell surface, a feature not observed in other cells of histiocytic origin [2].

Langerhans cells are normally found in the dermis, the reticuloendothelial system, the lung, and the pleura. They can be found in association with cigarette smoking in otherwise healthy individuals and can also be seen in other pulmonary disorders, such as idiopathic pulmonary fibrosis [8,9]. (See "Pathogenesis of idiopathic pulmonary fibrosis".)

In pulmonary Langerhans cell histiocytosis, the Langerhans cells are characteristically found in clusters and significantly outnumber those seen in other lung diseases. However, quantitative guidelines for the diagnosis of pulmonary Langerhans cell histiocytosis have not been established.

                

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Literature review current through: Nov 2016. | This topic last updated: Thu Dec 03 00:00:00 GMT+00:00 2015.
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