CTICasesEnd-stage Langerhans Cell Histiocytosis (LCH) with Severe Pulmonary Hypertension
Imaging findings
Radiograph shows large pulmonary arteries, right ventricular enlargement, and a busy, reticular-appearing lung parenchyma. CT reveals diffuse emphysema-like changes with discrete, thin-walled cysts of varying sizes throughout the lungs. Notably, the pulmonary arteries are quite large, and the vessels are preserved, contrasting with the diminutive vessels typically seen in bland emphysema. Severe pulmonary hypertension is evident by right ventricular compression of the left ventricle and right atrial enlargement. Cysts are sparse at the lung bases.
Key takeaways
End-stage Langerhans Cell Histiocytosis (LCH) can mimic emphysema but is characterized by discrete cysts and relatively preserved pulmonary vasculature, particularly in the lower lobes. Severe pulmonary hypertension, often out of proportion to the degree of lung destruction, is a recognized and poor prognostic feature of LCH, leading to right heart failure.
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