GENERALIZED IGG-LAMBDA PLASMACYTOMA AND C IMINO SHUNT STENOSIS CAUSEDBY PLASMA-CELL INFILTRATION

A 56-year-old apparently healthy man was on routine examination found to have a paraproteinaemia, classified as an IgG-lambda plasmocytoma i n stage 1 on the basis of a raised IgG level (1950 mg/dl) and partly b inuclear plasma cells in bone marrow. When 5 months later the IgG conc entration had increased to 2980 mg/dl and the proportion of plasma cel ls in bone marrow to 15%, treatment was begun with mephalan (0.25 mg/k g) and prednisolone (2 mg/kg), both on 4 successive days every 6 weeks . The patient's general condition rapidly worsened after 6 months. The extent of osteolyses increased, necessitating radiotherapy of the ver tebrae as well as a change in treatment to vincristin, cyclophosphamid e, doxorubicine and prednisolone. At that time renal failure set in. B ut the prophylactically placed Cimino shunt had become infiltrated wit h plasma cells causing shunt stenosis and soft-tissue swelling. The in filtration was reduced by irradiation with 18 Gy. Numerous bluish skin discolorations now appeared and plasma cell infiltrates were shown in lung and pleura, as well as in the abdomen. The patient died 15 month s after the diagnosis had been made.