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Pulmonary Langerhans Cell Histiocytosis (PLCH)

Imaging findings

High-resolution chest CT shows upper-lung zone predominant, irregular, thick- and thin-walled cysts with surrounding small nodular opacities, sparing the lung bases. These findings are highly characteristic of pulmonary Langerhans cell histiocytosis, which was confirmed on open lung biopsy.

Key takeaways

Pulmonary Langerhans cell histiocytosis is a smoking-related interstitial lung disease characterized by a combination of centrilobular nodules, cavitary nodules, and irregular thin-walled cysts with an upper-to-mid lung predominance. In patients who require lung transplantation but decline blood transfusions (such as Jehovah's Witnesses), complex multidisciplinary coordination and blood-conservation strategies are necessary.

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