Resolution of Primary Amyloidosis During Chemotherapy. Studies in a Patient with Nephrotic Syndrome

A patient with primary amyloidosis with evidence for a plasma cell dyscrasia but no abnormal immunoglobulin components had nephrotic syndrome with severe renal impairment. Kidney and bone marrow had extensive amyloid infiltration. She was treated with penicillamine, malphalan, prednisone, and fluoxymesterone; through 6 months renal function gradually improved; urine protein excretion dropped dramatically, serum albumin rose; liver size decreased; the bone marrow returned towards normal. During the next 4 1/2 years melphalan, prednisone, and fluoxymesterone treatment was continued with further improvement in renal function to normal levels. The morphologic characteristics and cellular relations of amyloid fibrils in the bone marrow were studied before, during, and after successful chemotherapy; the findings are evidence for a dual role for the reticuloendothelial cell in the formation and destruction of primary amyloidosis. This patient's response suggests that a multi-agent chemotherapy approach should be further studied.