Thrombotic Thrombocytopenic Purpura Medication
- Author: Theodore Wun, MD, FACP; Chief Editor: Emmanuel C Besa, MD more...
Medication Summary
Generally, drug therapy is reserved for refractory patients. Some hematologists routinely treat patients with steroids, and given the data that acquired TTP may be an autoimmune disorder with an inhibitory antibody to vWF-cleaving protease, this practice has appeal. The chemotherapeutic agent vincristine has been used as an adjunct to plasma exchange in patients with refractory disease, but its routine use has not been validated. Recent case reports have suggested that cyclosporin may be beneficial in patients with refractory disease even though this drug has been incriminated as a potential etiology of TTP.[5] Although used in the past, aspirin and dipyridamole are no longer used in treating TTP.
The anti-CD20 monoclonal antibody rituximab has also been reported to have activity in patients' refractory to plasma exchange. A study by Scully et al found that weekly rituximab given within 3 days of acute admission for TTP was safe and effective, with reduced stay and relapse.[6]
Chemotherapy agents
Class Summary
These agents are used as an adjunct to plasma exchange.
Vincristine (Oncovin, Vincasar)
Mechanism of action uncertain. May involve a decrease in reticuloendothelial cell function or increase in platelet production. However, neither of these mechanisms fully explains the effect in TTP and HUS.
Corticosteroids
Class Summary
These agents are used to treat idiopathic and acquired autoimmune disorders. They are also used as an adjunct to plasma exchange.
Prednisone (Sterapred)
May work by decreasing activity of reticuloendothelial system. In light of the evidence that patients with acquired TTP have an inhibitor to vWF-cleaving protease, steroids may decrease production of autoantibody.
Monoclonal antibody
Class Summary
These agents have shown efficacy in the treatment of autoimmune disorders.
Rituximab (Rituxan)
Anti-CD20 chimeric monoclonal antibody initially approved for therapy of follicular lymphoma. Has been shown to have activity in several autoimmune disorders such as immune thrombocytopenia, systemic lupus erythematosus, autoimmune hemolytic anemia, and rheumatoid arthritis.
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