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

Intern Med. 2014;53(17):1991-5. doi: 10.2169/internalmedicine.53.2065. Epub 2014 Sep 1.

Abstract

A 52-year-old woman with a high serum alkaline phosphatase (ALP) level underwent a liver biopsy, which showed diffuse heavy deposition of Aκ 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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyloidosis / diagnosis
  • Amyloidosis / metabolism
  • Amyloidosis / therapy*
  • Antineoplastic Agents, Alkylating / administration & dosage
  • Biopsy
  • Combined Modality Therapy
  • Diagnosis, Differential
  • Dose-Response Relationship, Drug
  • Female
  • Humans
  • Immunoglobulin Light Chains / metabolism*
  • Immunoglobulin Light-chain Amyloidosis
  • Liver / metabolism
  • Liver / pathology*
  • Melphalan / administration & dosage*
  • Middle Aged
  • Remission Induction
  • Stem Cell Transplantation*
  • Tomography, X-Ray Computed
  • Transplantation, Autologous

Substances

  • Antineoplastic Agents, Alkylating
  • Immunoglobulin Light Chains
  • Melphalan