Pulmonary Langerhans cell histiocytosis

Case contributed by Liz Silverstone
Diagnosis almost certain

Presentation

Fall with suspected rib fractures.

Patient Data

Age: 45 years
Gender: Male

Innumerable pulmonary cysts with upper zone predominance, variable size and shape, some internal structures, sparing the costophrenic recesses. Apical bullae.

MinIPs demonstrate a pattern of centrilobular cysts with a linear arrangement, an occasional branching pattern and subpleural sparing.

Case Discussion

Ex-smoker with 40 pack year history. Multiple pneumothoraces treated by pleurodesis and pleurectomy 15 years ago. Diagnosis of pulmonary Langerhans cell histiocytosis made elsewhere.

The airway arrangement of cystic destruction is not surprising because the walls of the terminal and respiratory bronchioles are infiltrated and destroyed by CD1a+ Langerhans-like cells 1,2.

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