title: [Fatal hepatic failure due to AL amyloidosis in a patient with multiple myeloma].
abstract: Although about 10 to 15% of patients with multiple myeloma (MM) develop AL amyloidosis, liver-restricted fatal amyloidosis is rare. We encountered such an MM patient. A 73-year-old female without a history of carpal tunnel syndrome was diagnosed with IgG-κ MM (Stage I by Durie & Salmon) in January, 2005. Because MM was exacerbated to Stage III in May, 2007, VAD (vincristine, adriamycin, dexamethasone) chemotherapy was performed with minor response, despite 3 courses of this regimen. Three courses of salvage chemotherapy (cyclophosphamide+melphalan; CM) were then performed with near partial response. In March, 2008, just before the 4th cycle of CM chemotherapy, she was slightly icteric with elevated biliary tract enzymes; therefore, treatment was switched to oral cyclophosphamide and prednisolone. At this time, she did not have macroglossia, skin eruption, gastrointestinal dysfunction, or bleeding. Echocardiography was also non-specific. One month later, she developed a marked bleeding tendency and leg edema. Laboratory tests showed a severe deterioration in liver function. In the middle of May, 2008, she progressed to hepatic coma and died of intracranial hemorrhage several days later. Autopsy showed that the liver was almost substituted by AL amyloid substance.
authors.affiliation: Department of Hematology and Clinical Immunology, Kobe City Medical Center General Hospital, Kobe, Japan.
authors.forename: Sumie
authors.initials: S
authors.lastname: Tabata
authors.forename: Masayuki
authors.initials: M
authors.lastname: Kurata
authors.forename: June
authors.initials: J
authors.lastname: Takeda
authors.forename: Yuuki
authors.initials: Y
authors.lastname: Funayama
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Yamauchi
authors.forename: Kazunari
authors.initials: K
authors.lastname: Aoki
authors.forename: Aiko
authors.initials: A
authors.lastname: Kato
authors.forename: Yuichirou
authors.initials: Y
authors.lastname: Ono
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Arima
authors.forename: Yoko
authors.initials: Y
authors.lastname: Takiuchi
authors.forename: Seiji
authors.initials: S
authors.lastname: Nagano
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsushita
authors.forename: Yukihiro
authors.initials: Y
authors.lastname: Imai
authors.forename: Takayuki
authors.initials: T
authors.lastname: Ishikawa
authors.forename: Takayuki
authors.initials: T
authors.lastname: Takahashi
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003907:Dexamethasone / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000150:complications; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D017093:Liver Failure / Q000209:etiology*; D008558:Melphalan / Q000627:therapeutic use; D009101:Multiple Myeloma / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D014750:Vincristine; D003907:Dexamethasone; D008558:Melphalan