[Primary amyloidosis with nephrotic syndrome: 5 year follow-up under polychemotherapy. A case report (author's...

A patient with primary amyloidosis had infiltration of liver, bone and bone marrow and a nephrotic syndrome with massive proteinuria. In addition, there was evidence for a plasma cell dyscrasia with an increased number of plasma cells and other lymphoreticular cell elements in the bone marrow and an impairment of immunoglobulin synthesis without M-gradient. The course of disease was observed over a period of five years. The patient was treated intermittently with a combination of penicillamine, melphalan, fluocortolone and fluoxymesterone. Under treatment the nephrotic syndrome disappeared and liver size decreased. Hemopoiesis remained sufficient but bone marrow infiltration by amyloid did not respond to therapy and massive osteoporosis developed. Comparison of our with other reported cases treated with the same regime suggests that response to this treatment may be influenced by factors such as sex and clinical manifestation of the disease.