Demographic features
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Peak age at presentation
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1-3 yrs [16]
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20-40 yrs [5]
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Smoking history
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Infrequently described [16, 17]
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Reported in > 95% [5, 12]
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Imaging findings; chest CT
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Distribution of abnormalities
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Frequently involves lower lobes [18]
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Sparing of bases and costo- phrenic angles is typical [19–21]
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Biological character
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Clonality
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Invariably reported [22–24]
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Suspect reactive rather than clonal [25]
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Clinical Presentation
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Single system vs multi-system presentation
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Typically part of multi-system LCH [16]
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Single system disease in > 80% of patients [5]
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Management
| | |
Pharmacotherapy with prednisone/vinblastine
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Complete or partial response frequently observed [26, 27]
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Insufficient data available; likely limited response
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Smoking cessation
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Limited role as tobacco exposure not involved in most cases
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Main and first line therapy in all adult smokers [28, 29]
|