Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Ann Intern Med. 2003 Jan 21;138(2):105-8.

Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab.

Author information

  • 1Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St. Louis, Missouri 63110, USA.

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.

Comment in

PMID:
12529092
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Silverchair Information Systems
    Loading ...
    Write to the Help Desk