AL amyloidosis with IgD-lambda monoclonal gammopathy and lambda-type Bence-Jones protein: successful treatment by autologous stem cell transplantation.
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.
Sakurai-Chin
Chanhyok
C
Nephrology Center, Toranomon Hospital Kajigaya, 1-3-1, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Ubara
Yoshifumi
Y
Suwabe
Tatsuya
T
Hoshino
Junichi
J
Yonaha
Tomoki
T
Hasegawa
Eiko
E
Sumida
Keiichi
K
Hiramatsu
Rikako
R
Yamanouchi
Masayuki
M
Hayami
Noriko
N
Yamauchi
Junji
J
Tominaga
Naoyuki
N
Sawa
Naoki
N
Takemoto
Fumi
F
Masuoka
Kazuhiro
K
Takaichi
Kenmei
K
Oohashi
Kenichi
K
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
D007072:Immunoglobulin D; D001536:Bence Jones Protein