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August 20, 1982

Diffuse Pulmonary Disease in a 2 1/2-Year-Old Child

JAMA. 1982;248(7):875-876. doi:10.1001/jama.1982.03330070063036

History  A 2 1/2 -year-old boy was first seen because of a nonproductive cough of two months' duration. A clinical diagnosis of bronchitis was made and the patient was discharged. Increasing dyspnea and fatigue during the subsequent three months precipitated further evaluation. The physical examination findings and laboratory data were unremarkable except for non-specific changes on auscultation of the chest. A chest roentgenogram was obtained (Figure) that demonstrates mild hyperaeration with predominately interstitial diffuse parenchymal infiltrates. "Cystic" areas are noted at the bases. The family relocated at this time. The child was examined at a distant medical center and treated for Mycoplasma infection without improvement. A radioisotope scan of the liver and spleen, a bone marrow biopsy specimen, and a radiological skeletal survey were normal. No additional physical signs were present. A lung biopsy was then performed.

Diagnosis  Primary pulmonary histiocytosis, often referred to as histiocytosis X.Microscopic examination of