Abstract
mogeneous light chain or Bence-Jones protein in urine and serum, plus the lack of characteristic “punched-out” radiolucencies in the skull radiograph should point toward the primary process. Despite current advances in understanding of the disease process, the treatment of primary amyloidosis has been less than satisfactory. Based on the fact that amyloid fibrils are derived from monoclonal light chains, the chemotherapeutic treatment of primary amyloidosis has been emphasized, with the use of alkylating agents such as melphalan, colchicine, or the combination of an alkylating agent and prednisone. In a study of 236 cases at the Mayo Clinic,4 the median survival after initial microscopic diagnosis was 14.7 months. Cardiac failure is the number one cause of death in 30% of the cases, followed by renal failure in 13%.
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