Nephrotic syndrome due to primary systemic AL amyloidosis, successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone.
We report 3 patients with nephrotic syndrome ascribed to primary systemic AL amyloidosis that were successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone. M-protein in serum disappeared soon after VAD, and nephrotic syndrome gradually improved in parallel with a decrease in daily protein excretion in urine. Long-term follow-up of these patients showed neither relapse of nephrotic syndrome nor reappearance of M-protein. High-dose melphalan followed by autologous stem cell support is a standard therapy for primary systemic AL amyloidosis, but in high-risk cases for this treatment, such as elderly patients and those with multiple organ involvement, VAD might be a therapeutic option.
Matsuda
Masayuki
M
Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto. matsuda@hsp.md.shinshu-u.ac.jp
Gono
Takahisa
T
Katoh
Nagaaki
N
Yoshida
Takuhiro
T
Tazawa
Ko-Ichi
K
Shimojima
Yasuhiro
Y
Ishii
Wataru
W
Fushimi
Tomohisa
T
Ikeda
Shu-Ichi
S
D000368:Aged; D000686:Amyloidosis / Q000150:complications*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003907:Dexamethasone / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin