AL amyloidosis that presented with marked hepatomegaly and polyclonal hypergammaglobulinemia

Abstract

A 65-year-old woman presented with marked hepatomegaly and polyclonal hypergammaglobulinemia. Her total serum protein was 8.9 g/dL with 35.6% albumin and 40.2% (35.8 mg/mL) γ globulin. Alkaline phosphatase was 730 IU/L. The cranio-caudal liver span measured on computed tomography was 24.2 cm. A biopsy of the liver revealed replacement of the liver parenchyma with amorphous eosinophilic materials that were stained positive with Congo red and showed the apple-green birefringence of amyloid; amyloid deposits were also observed in the gastric mucosa and bone marrow (BM). Although immunofixation of the serum and urine detected no monoclonal component, the serum free light chain (FLC) assay revealed an excess