title: Remission of chronic thrombotic thrombocytopenic purpura after treatment with cyclophosphamide and rituximab.
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.
  
  
  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.
authors.affiliation: Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St. Louis, Missouri 63110, USA.
authors.forename: Xinglong
authors.initials: X
authors.lastname: Zheng
authors.forename: Arnel M
authors.initials: AM
authors.lastname: Pallera
authors.forename: Lawrence T
authors.initials: LT
authors.lastname: Goodnough
authors.forename: J Evan
authors.initials: JE
authors.lastname: Sadler
authors.forename: Morey A
authors.initials: MA
authors.lastname: Blinder
mesh_terms: 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
chemical_list: 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