Remission of Chronic Thrombotic Thrombocytopenic Purpura after Treatment with Cyclophosphamide and Rituximab

  1. Xinglong Zheng, MD, PhD;
  2. Arnel M. Pallera, MD;
  3. Lawrence T. Goodnough, MD;
  4. J. Evan Sadler, MD, PhD; and
  5. Morey A. Blinder, MD
  1. From Washington University School of Medicine, St. Louis, Missouri.

    Abstract

    Background: Thrombotic thrombocytopenic purpura (TTP) in adults is usually caused by autoantibody inhibitors of ADAMTS13. Treatment with plasma exchange is often effective but does not address the underlying autoimmune process.

    Objective: To report the efficacy of intensive immunosuppressive therapy in refractory TTP.

    Design: Case report.

    Setting: University medical center.

    Patient: 42-year-old woman with chronic relapsing TTP.

    Intervention: Immunosuppression therapy with rituximab and cyclophosphamide.

    Measurements: ADAMTS13 activity and inhibitors and hematologic variables for TTP.

    Results: For 19 months, the patient had relapsing thrombotic microangiopathy despite plasma exchange; splenectomy; and therapy with vincristine, prednisone, and cyclosporine. ADAMTS13 activity was low, and tests detected an IgG inhibitor that recognized the metalloprotease domain of recombinant ADAMTS13. After treatment with rituximab and cyclophosphamide, the disease remitted, ADAMTS13 levels normalized, and the inhibitor was undetectable. The patient has required no treatment for 13 months.

    Conclusion: Intensive immunosuppressive therapy can lead to sustained clinical remission in patients with refractory autoimmune TTP.

    Article and Author Information

    • Acknowledgment: The authors thank Vanessa Thomas, RN, for assistance in caring for the patient.

    • Grant Support: By Howard Hughes Medical Institute and the National Institutes of Health (NHLBI T32 HL07088).

    • Potential Financial Conflicts of Interest:Expert testimony: L.T. Goodnough.

    • Requests for Single Reprints: Morey A. Blinder, MD, Washington University School of Medicine, 660 South Euclid Avenue, Box 8125, St. Louis, MO 63110; e-mail, blinder{at}labmed.wustl.edu.

    • Current Author Addresses: Drs. Zheng and Sadler: Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St. Louis, MO 63110.

    • Drs. Pallera and Blinder: Washington University School of Medicine, 660 South Euclid Avenue, Box 8125, St. Louis, MO 63110.

    • Dr. Goodnough: Washington University School of Medicine, 660 South Euclid Avenue, Box 8118, St. Louis, MO 63110.

    • Author Contributions: Conception and design: X. Zheng, A.M. Pallera, M.A. Blinder.

    • Analysis and interpretation of the data: X. Zheng, A.M. Pallera, L.T. Goodnough, J.E. Sadler, M.A. Blinder.

    • Drafting of the article: X. Zheng, A.M. Pallera, J.E. Sadler, M.A. Blinder.

    • Critical revision of the article for important intellectual content: X. Zheng, A.M. Pallera, L.T. Goodnough, J.E. Sadler, M.A. Blinder.

    • Final approval of the article: A.M. Pallera, L.T. Goodnough, J.E. Sadler, M.A. Blinder.

    • Provision of study materials or patients: X. Zheng, M.A. Blinder.

    • Obtaining of funding: J.E. Sadler.

    • Administrative, technical, or logistic support: A.M. Pallera, L.T. Goodnough.

    • Collection and assembly of data: X. Zheng, A.M. Pallera, M.A. Blinder.

    Summary for Patients

    • Summaries for Patients:Treatment of Refractory Thrombotic Thrombocytopenic Purpura Ann Intern Med January 21, 2003 138:I-38
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    1. Ann Intern Med January 21, 2003 vol. 138 no. 2 105-108
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