Pulmonary Langerhans Cell Histiocytosis Radiology
Etiology langerhans cell histiocytosis lch is an uncommon disease. Pulmonary langerhans cell histiocytosis plch is proliferation of monoclonal langerhans cells in lung interstitium and airspaces.
Adult Pulmonary Langerhans Cell Histiocytosis Thorax
It is caused by a disorder of myeloid dendritic cells.
Pulmonary langerhans cell histiocytosis radiology. Pulmonary langerhans cell histiocytosis is usually identified in young adults 20 40 years of age. A history of current or previous cigarette smoking is identified in up to 95 of cases 1 4 it is a rare disorder with no well established gender predilection which appears to be more common in caucasian populations 4. It most commonly appears in toddlers and very young children but you can.
There is a mid and upper zone predilection 1 3 4. Initial and final ct scans of 21 patients with lch and ct evidence of pulmonary disease were compared retrospectively. Pulmonary langerhans cell histiocytosis plch is an uncommon disorder of adult smokers associated with a significant morbidity.
Symptoms are dyspnea cough fatigue and pleuritic chest pain. One of these rare disorders which resembles some types of cancer is called langerhans cell histiocytosis or lch. Etiology is unknown but cigarette smoking plays a primary role.
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. 13 15 companion case and approach to imaging of cystic lung disease loading. The previously used term histiocytosis x included several entities with similar morphologic but different clinical manifestations.
Although langerhans cell histiocytosis lch is a familiar entity to most radiologists and to pediatric radiologists in particular it is but one of a group of disorders caused by the overproduction of histiocytes a subtype of white blood cells. Diagnosis is based on history and imaging tests and sometimes on bronchoalveolar. Several synonyms including histiocytosis x eosinophilic granuloma and langerhans cell granulomatosis have been used in the past but the term langerhans cell histiocytosis is now preferred.
Pulmonary langerhans cell histiocytosis plch previously called eosinophilic granuloma of the lung pulmonary langerhans cell granulomatosis and pulmonary histiocytosis x is an uncommon cystic interstitial lung disease that primarily affects young adults. 8 40 case findings of pulmonary langerhans cell histiocytosis on computed tomography. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease ranging from small peribronchiolar nodular opacities to multiple irregularly shaped cysts.
To document the evolution of pulmonary lesions of langerhans cell histiocytosis lch with sequential computed tomography ct.
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