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


METHODS
Case report.


METHODS
University medical center.


METHODS
42-year-old woman with chronic relapsing TTP.


METHODS
Immunosuppression therapy with rituximab and cyclophosphamide.


METHODS
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.


CONCLUSIONS
Intensive immunosuppressive therapy can lead to sustained clinical remission in patients with refractory autoimmune TTP.
Zheng
Xinglong
X
Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St. Louis, Missouri 63110, USA.
Pallera
Arnel M
AM
Goodnough
Lawrence T
LT
Sadler
J Evan
JE
Blinder
Morey A
MA
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D000328:Adult; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies / Q000276:immunology; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008666:Metalloendopeptidases / Q000172:deficiency / Q000276:immunology; D010976:Platelet Count; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy* / Q000276:immunology; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D014841:von Willebrand Factor / Q000276:immunology
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D007166:Immunosuppressive Agents; D014841:von Willebrand Factor; D000069283:Rituximab; D003520:Cyclophosphamide; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human