Pulmonary Langerhans Cell Histiocytosis
OVERVIEW
Proliferation of monoclonal CD1a-positive Langerhans cells that infiltrate the bronchioles and alveolar interstitium, along with lymphocytes, plasma cells, neutrophils, and eosinophils.1,2
EPIDEMIOLOGY1
Rare disease but prevalence and incidence cannot be determined due to lack of population-based studies.
- The 5-year survival rate is 74%
- Median survival is 12 years
RISK FACTORS1,2
- Cigarette smoking plays a primary role
- Men and women affected equally
- Patients are at increased risk of cancer
The typical patient is a white smoker and 20 to 40 years old.1,2
