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: James N George
• Journal: American journal of hematology (Am J Hematol) Vol. 87 Suppl 1 Pg. S88-91 (May 2012) ISSN: 1096-8652 [Electronic] United States
• PMID: 22407740 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2012 Wiley Periodicals, Inc.

Chemical References

• Adrenal Cortex Hormones
• Antibodies, Monoclonal, Murine-Derived
• Immunologic Factors
• Recombinant Proteins
• von Willebrand Factor
• Rituximab
• ADAM Proteins
• ADAMTS13 Protein
• ADAMTS13 protein, human

Topics

• ADAM Proteins (blood, therapeutic use)
• ADAMTS13 Protein
• Adrenal Cortex Hormones (therapeutic use)
• Antibodies, Monoclonal, Murine-Derived (therapeutic use)
• Catheterization, Central Venous
• Disease-Free Survival
• Humans
• Immunologic Factors (therapeutic use)
• Plasma Exchange
• Purpura, Thrombotic Thrombocytopenic (blood, mortality, therapy)
• Recombinant Proteins (blood, therapeutic use)
• Recurrence
• Remission Induction
• Rituximab
• Survival Rate
• von Willebrand Factor (antagonists & inhibitors, metabolism)