Therapeutic Outcome of Cyclic VAD (Vincristine, Doxorubicin and Dexamethasone) Therapy in Primary Systemic AL Amyloidosis Patients

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  • Tazawa Ko-ichi
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Matsuda Masayuki
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Yoshida Takuhiro
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Gono Takahisa
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Katoh Nagaaki
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Shimojima Yasuhiro
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Ishii Wataru
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Fushimi Tomohisa
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine
  • Koyama Jun
    Department of Medicine (Cardiology), Shinshu University School of Medicine
  • Ikeda Shu-ichi
    Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine

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Objective Intensive chemotherapy targeting plasma cell dyscrasia has been recently employed for the treatment of primary systemic AL amyloidosis. We prospectively studied the clinical usefulness of cyclic VAD (vincristine, doxorubicin and dexamethasone) in patients with primary systemic AL amyloidosis who were ineligible for high-dose melphalan with autologous stem cell support.<br> Patients and Methods Eight patients (mean age, 60.4±8.8 years) were treated with cyclic VAD until the disappearance of M-protein from both serum and urine. Of these, seven showed nephrotic syndrome before the start of VAD irrespective of a decrease in creatinine clearance. Serial follow-up studies after VAD evaluated hematological status and organ function.<br> Results Four patients (50%) showed a marked decrease in abnormal plasma cells in the bone marrow and normalized κ/λ ratios of serum free light chain in conjunction with disappearance of M-protein after 1 to 3 courses of VAD. There were no serious adverse events, and nephrotic syndrome gradually improved with no hematological relapse in the follow-up period of 3 to 5 years. The remaining 4 patients showed worsening of congestive heart failure and/or systemic edema ascribable to dexamethasone, resulting in cessation of cyclic VAD before disappearance of M-protein. All of these patients died of multiple organ failure or required permanent hemodialysis within 1 year after the start of cyclic VAD.<br> Conclusion Cyclic VAD is a potent therapeutic option in primary systemic AL amyloidosis, but in patients with renal or cardiac dysfunction careful management for adverse events, especially body fluid retention, is necessary.<br>

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  • Internal Medicine

    Internal Medicine 47 (17), 1517-1522, 2008

    一般社団法人 日本内科学会

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