title: Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura.
abstract: Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to further increase survival as well as decrease the frequency of relapse. With adjuvant treatment, durable remissions can be achieved more quickly, requiring fewer days of plasma exchange. Fewer days of plasma exchange have resulted in fewer complications, such as central venous catheter-related systemic infections. Future potential options for adjuvant treatment, recombinant ADAMTS13 to correct severe ADAMTS13 deficiency, and agents to block von Willebrand factor-mediated platelet thrombosis are being investigated.
authors.affiliation: Department of Biostatistics and Epidemiology, College of Public Health, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73190, USA. james-george@ouhsc.edu
authors.forename: James N
authors.initials: JN
authors.lastname: George
mesh_terms: D051722:ADAM Proteins / Q000097:blood / Q000627:therapeutic use; D000071120:ADAMTS13 Protein; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002405:Catheterization, Central Venous; D018572:Disease-Free Survival; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000401:mortality / Q000628:therapy*; D011994:Recombinant Proteins / Q000097:blood / Q000627:therapeutic use; D012008:Recurrence; D012074:Remission Induction; D000069283:Rituximab; D015996:Survival Rate; D014841:von Willebrand Factor / Q000037:antagonists & inhibitors / Q000378:metabolism
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D011994:Recombinant Proteins; D014841:von Willebrand Factor; D000069283:Rituximab; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human