title: AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation.
abstract: A 45-year-old Japanese woman had been diagnosed with monoclonal gammopathy of undetermined significance (MGUS) featuring urinary Bence-Jones protein of the lambda type (BJP-lambda) for 11 years. She then developed eyelid purpura, dyspnea, and flank pain. Abdominal CT scans revealed renal infarction. Biopsy of the kidney, heart, jejunum, and skin demonstrated amyloid deposits in the vessel walls, but not in the glomeruli. She was diagnosed as having AL amyloidosis with IgD-lambda monoclonal gammopathy and BJP-lambda. Autologous stem cell transplantation (SCT) was done after chemotherapy with vincristine, daunorubicin, dexamethasone (VAD), and high-dose melphalan (HDM). This reduced the IgD level from 156 to 0.1 mg/dL, along with the disappearance of BJP, despite cerebral infarction during chemotherapy. We recommend SCT for patients with IgD-associated AL amyloidosis.
authors.affiliation: Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
authors.forename: Chanhyok
authors.initials: C
authors.lastname: Sakurai-Chin
authors.forename: Yoshifumi
authors.initials: Y
authors.lastname: Ubara
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Suwabe
authors.forename: Junichi
authors.initials: J
authors.lastname: Hoshino
authors.forename: Tomoki
authors.initials: T
authors.lastname: Yonaha
authors.forename: Eiko
authors.initials: E
authors.lastname: Hasegawa
authors.forename: Keiichi
authors.initials: K
authors.lastname: Sumida
authors.forename: Rikako
authors.initials: R
authors.lastname: Hiramatsu
authors.forename: Masayuki
authors.initials: M
authors.lastname: Yamanouchi
authors.forename: Noriko
authors.initials: N
authors.lastname: Hayami
authors.forename: Junji
authors.initials: J
authors.lastname: Yamauchi
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Tominaga
authors.forename: Naoki
authors.initials: N
authors.lastname: Sawa
authors.forename: Fumi
authors.initials: F
authors.lastname: Takemoto
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Masuoka
authors.forename: Kenmei
authors.initials: K
authors.lastname: Takaichi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Oohashi
mesh_terms: D000686:Amyloidosis / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D001536:Bence Jones Protein / Q000652:urine*; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007072:Immunoglobulin D / Q000276:immunology*; D008875:Middle Aged; D010265:Paraproteinemias / Q000276:immunology / Q000473:pathology / Q000503:physiopathology / Q000601:surgery*; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D007072:Immunoglobulin D; D001536:Bence Jones Protein