Answer BOF 31

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BOF: 31

A patient who is known to have restrictive lung disease and a chest x-ray that shows reticular shadowing has an open lung biopsy. The pathologist reports the presence of cells with pentalaminar inclusions in their cytoplasm (Birbeck granules, X bodies)

This is diagnostic of:

a)      Sarcoidosis

b)      Idiopathic fibrosing alveolitis

c)      Asbestosis

d)      Lymphangioleiomyoatosis

e)      Langerhans’ cell histiocytosis

Answer:

e)

Isolated pulmonary involvement may occur in Langerhans’ cell histiocytosis (Histiocytosis X) and is confirmed by the finding of Birbeck granules within the Langerhans’ cells. Langerhans’ cell histiocytosis refers to a clonal proliferation of Langerhans’ cells which are histiocytes found in the dermis.

Last Updated: 16/04/06

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