title: [Improvement of hepatomegaly after treatment with autologous hematopoietic stem cell transplantation followed by bortezomib and dexamethasone in Bence-Jones protein κ-type of multiple myeloma with systemic amyloidosis].
abstract: A 51-year-old man was admitted to our hospital complaining of right upper quadrant abdominal and back pain. Severe hepatomegaly (six fingerbreadths) was detected by liver palpation. Blood test results showed cholestatic liver disease. He was diagnosed with amyloidosis by liver biopsy. Bone marrow aspiration revealed 15% of contents to be plasma cells. BJPκ was detected by urine electrophoresis. Therefore, he was diagnosed with the BJPκ type of multiple myeloma with systemic amyloidosis. The patient was treated with bortezomib, dexamethasone and high-dose melphalan with autologous peripheral blood stem cell transplantation. He achieved VGPR (very good partial response) after transplantation. Hepatomegaly improved but swelling persisted, and he was therefore treated with 1.3 mg/m(2)/day of bortezomib and 20 mg/day of dexamethasone on day 1 and day 15 in 28-day cycles. Upon finishing 22 cycles in June 2014, his liver had returned to normal size. Restoration of normal liver size after treatment is rare in cases with severe hepatomegaly due to systemic amyloidosis. We thus report our case with a review of the relevant literature.
authors: Department of Hematology, Sapporo Kiyota Hospital.
authors: Yasuhiro
authors: Y
authors: Nagamachi
authors: Naofumi
authors: N
authors: Yamauchi
authors: Hirohito
authors: H
authors: Muramatsu
authors: Naoya
authors: N
authors: Miyajima
authors: Hidetoshi
authors: H
authors: Inomata
authors: Tetsuro
authors: T
authors: Okamoto
authors: Eri
authors: E
authors: Nozawa
authors: Yoshirou
authors: Y
authors: Gotoh
authors: Ryuzo
authors: R
authors: Koyama
authors: Koji
authors: K
authors: Ihara
authors: Takuji
authors: T
authors: Nishisato
authors: Junji
authors: J
authors: Kato
mesh_terms: D000686:Amyloidosis / Q000150:complications / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001536:Bence Jones Protein / Q000032:analysis; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003907:Dexamethasone / Q000008:administration & dosage; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D000075363:Immunoglobulin Light-chain Amyloidosis; D008297:Male; D008875:Middle Aged; D009101:Multiple Myeloma / Q000150:complications / Q000628:therapy*; D011719:Pyrazines / Q000008:administration & dosage; D014182:Transplantation, Autologous
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D003907:Dexamethasone; D001536:Bence Jones Protein