부산대학교 도서관

A 64 year-old case of primary diffuse alveoloseptal amyloidosis was presented. Serial chest roentgenograms revealed slowly progressive deterioration of small nodular pattern during seven years. The diagnosis of pulmonary amyloidosis was obtained by transbronchial lung biopsy. A bone marrow examination was normal, and a rectal biopsy showed no evidence of amyloid. Immunoelectrophoresis showed an increase of IgM of λ type. He deteriorated gradually with increasing dyspnea and died in 1982.At autopsy the lungs presented an expanded appearance without any tendency toward the usual postmortem collapse. On section the cut surfaces of the lungs had a granular appearance and resembled a fruit cake crumbling to pieces. There were diffuse amyloid infiltration of alveolar septa and some amyloid deposits in the walls of bronchioles and pulmonary small vessels. Small diffuse calcified nodules were found. Amyloid deposition was also found in hilar and mediastinal lymph nodes and the spleen, but there was no amyloid deposit in the heart, intestines, tongue or brain. To our knowledge, this is the twentieth reported case of primary diffuse pulmonary amyloidosis.