Marked and Rapid Regression of Hepatic Amyloid Deposition in a Patient with Systemic Light Chain (AL) Amyloidosis after High-dose Melphalan Therapy with Stem Cell Transplantation

摘要

A 52-year-old woman with a high serum alkaline phosphatase (ALP) level underwent a liver biopsy, which showed diffuse heavy deposition of A. amyloid, and was diagnosed as having immunoglobulin light chain (AL) amyloidosis. Although she received high-dose melphalan with stem cell transplantation and achieved a hematologic complete response (CR), her ALP level began to increase one year after treatment. Further examinations revealed that she was still in a CR state with dominant bone-type ALP, and re-biopsied liver specimens demonstrated marked regression of amyliod deposition, providing important evidence that the turnover of hepatic amyloid proteins can actually occur more rapidly than previously thought.