title: 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.
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.
authors.affiliation: Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Japan.
authors.forename: Nagaaki
authors.initials: N
authors.lastname: Katoh
authors.forename: Akira
authors.initials: A
authors.lastname: Matsushima
authors.forename: Masahiro
authors.initials: M
authors.lastname: Kurozumi
authors.forename: Masayuki
authors.initials: M
authors.lastname: Matsuda
authors.forename: Shu-ichi
authors.initials: S
authors.lastname: Ikeda
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy*; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage; D001706:Biopsy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D000075363:Immunoglobulin Light-chain Amyloidosis; D008099:Liver / Q000378:metabolism / Q000473:pathology*; D008558:Melphalan / Q000008:administration & dosage*; D008875:Middle Aged; D012074:Remission Induction; D033581:Stem Cell Transplantation; D014057:Tomography, X-Ray Computed; D014182:Transplantation, Autologous
chemical_list: D018906:Antineoplastic Agents, Alkylating; D007147:Immunoglobulin Light Chains; D008558:Melphalan