An autopsied case of primary amyloidosis associated with diffuse pulmonary amyloid deposition and amyloid arthropathy with bone destruction.

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A 63-year-old man, who had sustained progressing polyarthropathy mimicing rheumatoid arthritis for the past 20 years, suffered from pathological fractures of the left and, 6 months later, of the right femoral neck. Histological examination revealed massive deposits of amyloid in the femoral necks and periarticular tissue. On bone X-ray films, osteolytic lesions were found in the left humeral head and in the left tibia. Atypical plasma cells moderately increased in number on bone marrow smears. One year after the initial fracture, the patient died of respiratory failure. At autopsy, massive deposits of amyloid were demonstrated in alveolar septa and blood vessels throughout the lungs. The type of the amyloid was AL. No pathologocal changes indicating multiple myeloma were noted in the bone marrow. The final diagnosis was primary amyloidosis associated with amyloid arthropathy and diffuse pulmonary amyloidosis. The latter seems to have led to adult respiratory distress syndrome as the major cause of death.
日本臨床免疫学会の論文