Fig. 16.1 Smooth interlobular septal thickening with lymphomatous involvement of the lungs in a 27-year-old woman with diffuse large B-cell lymphoma. (a, b) Lung window of CT scans (5.0-mm section thickness) obtained at levels of bronchus intermedius (a) and suprahepatic superior vena cava (b), respectively, shows diffuse ground-glass opacity with smooth interlobular septal thickening (arrows) in both lungs, particularly in anterior lungs. Also note enlarged lymph nodes in bilateral hila (open arrows in a) and in subcarcinal area (arrowhead in a). (c) Coronal reformatted image (5.0-mm section thickness) also

demonstrates diffuse ground-glass opacity with smooth interlobular septal thickening in both lungs. (d) Low-magnification (×40) photomicrograph of pathologic specimen obtained from right upper lobe from a different patient (a 52-year-old man with marginal zone B-cell lymphoma) discloses smooth interlobular septal thickening (arrows). Also note thickened alveolar walls. In this patient, thin-section CT scan showed localized area of ground-glass opacity containing internal smooth interlobular septal thickening (not shown here)

Pharmacologic measures to reduce edema accumulation, such as with diuretics, inotropic agents, and vasodilators, can be used.

lipid-storing foam histiocytes, usually seen within the alveoli, the alveolar walls, and the lymphatic interlobular and subpleural spaces, while the pulmonary architecture remains normal [2].

Niemann–Pick Disease

Pathology and Pathogenesis

Niemann–Pick types A and B (NPA and NPB), also called acid sphingomyelinase deficiency, are caused by the deficiency of a specific enzyme, acid sphingomyelinase. The characteristic microscopic feature is an infiltration of

Symptoms and Signs

The adult form of Niemann–Pick disease (NPB), which appears during the second and third decades, is relatively benign, presenting with hepatosplenomegaly, hemostatic defects, and, occasionally, cerebellar ataxia. Interstitial lung disease is usually asymptomatic [9].