AL amyloidosis that presented with marked hepatomegaly and polyclonal hypergammaglobulinemia

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  • 顕著な肝腫大と多クローン性高γグロブリン血症で発症したALアミロイドーシスの1例

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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 of FLC-κ (FLC-κ, 1,290 mg/L; FLC-λ, 86 mg/L). The BM contained 10.4% clonal plasma cells carrying the CCND1-immunoglobulin heavy chain fusion gene. She was diagnosed with AL amyloidosis and treated with bortezomib-based chemotherapy, readily leading to the hematological response fulfilling the criteria of very good partial response. The hepatomegaly was steadily resolved in response to persistent administration of bortezomib for >2 years. It is possible that the hypergammaglobulinemia reflected a reactive process against amyloid deposits in the liver. This report suggests that plasma cell-targeting therapy can reduce the amyloid deposits from the involved organs, potentially reversing their dysfunction.

Journal

  • Tenri Medical Bulletin

    Tenri Medical Bulletin 20 (1), 63-72, 2017

    Tenri Foundation, Tenri Institute of Medical Research

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