|
Demographic features
| | |
|
Peak age at presentation
|
1-3 yrs [16]
|
20-40 yrs [5]
|
|
Smoking history
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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 [19–21]
|
|
Biological character
| | |
|
Clonality
|
Invariably reported [22–24]
|
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]
|
