Primary systemic amyloidosis: resolution of the nephrotic syndrome with melphalan and prednisone

Abstract

We describe two patients with primary systemic amyloidosis (AL) and the nephrotic syndrome. The administration of melphalan and prednisone was associated with resolution of the nephrotic syndrome. The serum albumin level returned to normal, proteinuria decreased to near normal, edema resolved, and the monoclonal protein in the serum and urine disappeared. In both patients, renal function remained stable and hepatomegaly disappeared. In both, however, amyloid deposition was greater in follow-up renal tissue than in the initial specimen. The effect of systemic therapy in AL must be assessed with histologic observations as well as clinical indexes.