Polyclonal plasma cell proliferation with systemic capillary hemangiomatosis, endocrine disturbance, and peripheral...

An autopsy case of so-called plasma cell dyscrasia (PCD) of a 37-year-old woman was reported. This case showed typical clinical symptoms of PCD, except for no abnormality in serum immunoglobulins. Besides the typical histological findings often observed with PCD, the autopsy revealed an additional finding of systemic angiomatous proliferation of small blood vessels throughout the body, especially in the skin, kidneys, brain, various lymph nodes, and soft tissue. A peculiar glomerular lesion in this case, similar to membranoproliferative glomerulonephritis, was likely to be a result of proliferation of glomerular capillaries. In addition, this case showed histological evidence of retroperitoneal fibrosis and osteosclerosis. These findings were also considered to be due to the vascular abnormality. Although the etiology of the vascular lesions is obscure, it is emphasized that the abnormality is probably an important cause of the clinical symptoms seen in PCD.