Successful treatment of primary AL amyloidosis by VAD therapy, high-dose melphalan, and autologous peripheral stem cell transplantation.
We report a 58-year-old Japanese man with primary systemic AL amyloidosis who achieved disappearance of proteinuria including Bence-Jones protein (lambda-type) after two courses of VAD therapy (vincristine, doxorubicin, and dexamethasone) and subsequent high-dose melphalan, followed by autologous peripheral blood stem cell transplantation. Because this patient did not have any apparent amyloidosis-related heart or liver damage and met all of the eligibility criteria for this therapy, this treatment was performed. Both proteinuria and M-protein disappeared completely, and he is doing well clinically at 19 months after treatment. However, amyloid deposits were still found in the kidneys, including the glomeruli and tubulointerstitium, when renal biopsy was done at 8 months after treatment. In the future, we may reach a time when clinical remission corresponds to histological remission.
Yamazaki
Osamu
O
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Ubara
Yoshifumi
Y
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan. ubara@toranomon.gr.jp.
Suwabe
Tatsuya
T
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Nakanishi
Shohei
S
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Hoshino
Junichi
J
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Sawa
Naoki
N
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Hayami
Noriko
N
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Yamanouchi
Masayuki
M
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Takemoto
Fumi
F
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Kenmei
Takaichi
T
Nephrology Center, Toranomon Hospital, 1-3-1 Kajigaya, Takatsu, Kawasaki, Kanagawa, 212-0015, Japan.
Masuoka
Kazuhiro
K
Department of Hematology, Toranomon Hospital, Kajigaya, Kawasaki, Kanagawa, Japan.
Oohashi
Kenichi
K
Department of Pathology, Toranomon Hospital, Kajigaya, Kawasaki, Kanagawa, Japan.
D000686:Amyloidosis / Q000473:pathology / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols; D003907:Dexamethasone; D004317:Doxorubicin; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007668:Kidney / Q000378:metabolism / Q000473:pathology; D008297:Male; D008558:Melphalan / Q000627:therapeutic use*; D008875:Middle Aged; D014750:Vincristine
D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D008558:Melphalan