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Progressive Langerhans Cell Histiocytosis (PLCH)

Progressive Langerhans Cell Histiocytosis (PLCH)▶ Watch on YouTube — age-restricted, cannot embed here

Imaging findings

Early findings (2010 radiograph) showed multifocal parenchymal opacities. Later (2011 CT), cystic changes and nodules (some cavitating) were seen, evolving into very severe and extensive lung disease by 2013 with extensive irregular cystic spaces. Airway narrowing was also observed, with thickened anterior airway walls and a narrow bronchus intermedius. Nodular lesions were also seen in the airways.

Key takeaways

A florid and progressive case of pulmonary Langerhans Cell Histiocytosis (PLCH) in a smoker. It beautifully demonstrates the evolution from early nodular and cystic lesions to severe, 'burned-out' disease, ultimately leading to transplant consideration. The case also raises the speculation of direct airway involvement by Langerhans cell histiocytosis given the observed airway narrowing and nodules.

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