Which medications are used in the treatment of thrombotic thrombocytopenic purpura (TTP)?

Updated: Feb 18, 2019
  • Author: Theodore Wun, MD, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

The therapy of choice is plasma exchange with fresh frozen plasma and immunosuppression.

In January 2019 the FDA approved caplacizumab (Cablivi) for adults with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy. It has been shown to reduce time to platelet count response and also to reduce aTTP-related death, recurrence, or major thromboembolic events. [5]

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. Case reports have suggested that cyclosporine may be beneficial in patients with refractory disease even though this drug has been incriminated as a potential trigger of TTP. [28] Although used in the past, aspirin and dipyridamole are no longer used in treating TTP.

The anti-CD20 monoclonal antibody rituximab has been reported to have activity in TTP that is 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. [29] A study by Froissart and colleagues, in 22 adult patients who had responded poorly to plasma exchange, found that treatment with rituximab resulted in shorter overall treatment duration and reduced 1-year relapses, compared with historical controls. [21]

Rituximab is typically given in a dosage of 375 mg/m2 weekly for 4 weeks. Ideally, at least 4 hours should elapse between administration of rituximab and plasma exchange. [15]


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