Pulmonary Langerhans Cell Histiocytosis Ct
Adult pulmonary langerhans cell histiocytosis is a rare disorder of unknown aetiology that occurs predominantly in young smokers with an incidence peak at 20 40 yrs of age. Your provider may order a ct scan a type of x ray of your chest.
Pulmonary Langerhans Cell Histiocytosis Imaging Appearance And
Initial and final ct scans of 21 patients with lch and ct evidence of pulmonary disease were compared retrospectively.
Pulmonary langerhans cell histiocytosis ct. In adults pulmonary involvement with langerhans cell histiocytosis usually occurs as a single system disease and is characterised by focal langerhans cell granulomas infiltrating and destroying distal bronchioles. Pulmonary langerhans cell histiocytosis plch is a rare interstitial granulomatous disease that usually affects young adults who are smokers. Pulmonary langerhans cell histiocytosis plch is a relatively uncommon lung disease that generally but not invariably occurs in cigarette smokers.
Pulmonary langerhans cell histiocytosis is usually identified in young adults 20 40 years of age. Diagnosing pulmonary langerhans cell histiocytosis. 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.
Chest computed tomography ct allows a confident diagnosis of plch only in typical presentation when nodules cavitated nodules and cysts coexist and predominate in the upper and middle lungs. There is a mid and upper zone predilection 1 3 4. It is caused by a disorder of myeloid dendritic cells.
High resolution chest computed tomography ct scan. To document the evolution of pulmonary lesions of langerhans cell histiocytosis lch with sequential computed tomography ct. The pathologic hallmark of plch is the accumulation of langerhans and other inflammatory cells in small.
It can be hard for health care providers to diagnose plch because people have different symptoms. 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. Pulmonary langerhans cell histiocytosis is a disease in which monoclonal cd1a positive langerhans cells a type of histiocyte infiltrate the bronchioles and alveolar interstitium accompanied by lymphocytes plasma cells neutrophils and eosinophils.
Provider can use the following tests to see if you have plch. Pulmonary langerhans cell histiocytosis has variable appearance depending on the stage of disease ranging from small peribronchiolar nodular opacities to multiple irregularly shaped cysts.
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