A role for ITPA variants in the clinical course of pulmonary Langerhans' cell histiocytosis?

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A role for ITPA variants in the clinical course of pulmonary Langerhans' cell histiocytosis?

Interstitial lung diseases (ILD) comprise a number of clinical conditions, including sarcoidosis, pulmonary fibrosis and pulmonary Langerhans’ cell histiocytosis (PLCH; histiocytosis X). The diagnostic workup for the classification of ILD is often complicated and tedious. PLCH is characterised by the proliferation of Langerhans’ cells and their infiltration into pulmonary tissues. In disseminat...

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Pulmonary langerhans cell histiocytosis

Pulmonary Langerhans' cell histiocytosis (PLCH) remains an important diagnostic consideration in the differential diagnosis of diffuse infiltrative lung disease, particular among smokers. This review highlights recent progress in our understanding of the etiology, clinical presentation, and diagnostic and therapeutic approaches to this unusual lung disorder. Evolving studies strongly link the b...

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Pulmonary Langerhans cell Histiocytosis.

Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocy...

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Langerhans cell histiocytosis

Introduction: Langerhans cell histiocytosis (LCH), which was previously known as histiocytosis X, refers to a group of lesions that stimulate uncontrolled proliferation of cells. The purpose of this case was to report and describe a LCH case with mandibular bone involvement in a 2.5-year-old boy. Case reports: A 2.5 years old boy with LCH of mandibular jaw who has been treated by surgical inter...

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ژورنال

عنوان ژورنال: European Respiratory Journal

سال: 2010

ISSN: 0903-1936,1399-3003

DOI: 10.1183/09031936.00026610