A 22-year-old heavy smoker has progressive exertional dyspnea and spontaneous pneumothorax. HRCT shows upper lobe thin walled cysts and stellate nodules with sparing of the costophrenic angles. Bronchoalveolar lavage cytology shows cells positive for CD1a and S100 with Birbeck granules on electron microscopy. What is the most appropriate management step?
- A Start corticosteroids and continue smoking
- B Begin empirical antituberculous therapy
- C Smoking cessation, which may lead to regression ✓
- D Refer for immediate lung transplantation
Correct answer: C. Smoking cessation, which may lead to regression
Explanation
This is pulmonary Langerhans cell histiocytosis, seen almost exclusively in young adult smokers, characterized by stellate bronchiolocentric nodules progressing to bizarre shaped cysts with costophrenic angle sparing. The lesional Langerhans cells express CD1a and S100 and contain Birbeck granules. Smoking cessation is the cornerstone of treatment and can produce regression; transplantation is reserved for advanced respiratory failure.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.