Nephrotic syndrome due to primary systemic AL amyloidosis, successfully treated with VAD (vincristine, doxorubicin and dexamethasone) alone |
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Authors: | Matsuda Masayuki Gono Takahisa Katoh Nagaaki Yoshida Takuhiro Tazawa Ko-Ichi Shimojima Yasuhiro Ishii Wataru Fushimi Tomohisa Ikeda Shu-Ichi |
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Affiliation: | Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto. matsuda@hsp.md.shinshu-u.ac.jp |
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Abstract: | 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. |
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