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Table 1 Contrasting pediatric and adult PLCH

From: Pulmonary langerhans cell histiocytosis

  Pediatric PLCH Adult PLCH
Demographic features   
Peak age at presentation 1-3 yrs [16] 20-40 yrs [5]
Smoking history Infrequently described [16, 17] Reported in > 95% [5, 12]
Imaging findings; chest CT   
Distribution of abnormalities Frequently involves lower lobes [18] Sparing of bases and costo- phrenic angles is typical [1921]
Biological character   
Clonality Invariably reported [2224] Suspect reactive rather than clonal [25]
Clinical Presentation   
Single system vs multi-system presentation Typically part of multi-system LCH [16] Single system disease in > 80% of patients [5]
Management   
Pharmacotherapy with prednisone/vinblastine Complete or partial response frequently observed [26, 27] Insufficient data available; likely limited response
Smoking cessation Limited role as tobacco exposure not involved in most cases Main and first line therapy in all adult smokers [28, 29]