Histiocytosis X
Histiocytosis X Causes
People with PLCH are generally cigarette smokers. Antigenic stimulation from 1 or more components of tobacco smoke is likely responsible for the onset of this disease. Because only a few tobacco smokers develop the disease, other susceptibility factors, such as host genetics and environmental exposures, most likely play a significant role in pathogenesis. Some reports in the literature also describe PLCH developing after radiation and/or chemotherapy for lymphoma.
Histiocytosis X Definition
Eosinophilic granuloma, also known as pulmonary histiocytosis X (PHX) or pulmonary Langerhans cell histiocytosis X (PLCH), is a rare interstitial lung disease that is epidemiologically related to tobacco smoking. It predominantly affects young adults, primarily occurring in the third or fourth decades of life.
Histiocytosis X Diagnosis
Chest x-rays, pulmonary function tests, and bronchoscopy with biopsy showing typical abnormalities in histiocytes are used to diagnose the problem.
Histiocytosis X Symptoms and Signs
Adult symptoms include: cough; shortness of breath; chest pain, fever; weight loss; general discomfort, uneasiness or ill feeling (malaise); increased amount or urine output; thirst and increased drinking of fluids; bone pain; and rash.
Histiocytosis X Treatment
This disorder is treated with corticosteroids, which inhibits immune function (including the dangerous cells). Smoking may exacerbate the response to treatment and should be stopped.