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Thrombotic Thrombocytopenic Purpura (TTP) Medication

  • Author: Theodore Wun, MD, FACP; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
 
Updated: Jun 20, 2016
 

Medication Summary

Generally, drug therapy in thrombotic thrombocytopenic purpura (TTP) is reserved for patients with refractory disease. Some hematologists routinely treat patients with steroids, and given the data that acquired TTP may be an autoimmune disorder with an inhibitory antibody to von Willebrand factor (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 cyclosporine may be beneficial in patients with refractory disease even though this drug has been incriminated as a potential trigger of TTP.[26] 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 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.[27] 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.[19]

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.[12]

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Blood Products

Class Summary

Plasma exchange with FFP is the therapy of choice for TTP.Fresh frozen plasma (FFP, Octaplas)

Fresh frozen plasma (FFP, Octaplas)

 

Plasma provides all plasma proteins and clotting factors to support adequate hemostasis to treat or prevent bleeding or to treat other protein deficiencies that cannot be replaced with protein specific concentrates. It is indicated for plasma exchange in patients with TTP. Octaplas is a solvent detergent treated, pooled FFP.

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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.

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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.

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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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Contributor Information and Disclosures
Author

Theodore Wun, MD, FACP Professor of Medicine, Professor of Pathology and Laboratory Medicine, University of California Davis School of Medicine; Chief of Hematology/Oncology, Program Director, Veterans Affairs Northern California Health Care System; Medical Director, University of California Davis CCRC

Theodore Wun, MD, FACP is a member of the following medical societies: American Association of Blood Banks, American Society for Blood and Marrow Transplantation, American College of Physicians, American Federation for Medical Research, American Society of Hematology, SWOG

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, SWOG

Disclosure: Partner received none from No financial interests for none.

Chief Editor

Srikanth Nagalla, MBBS, MS, FACP Director, Clinical Hematology, Cardeza Foundation for Hematologic Research; Assistant Professor of Medicine, Division of Hematology, Associate Program Director, Hematology/Medical Oncology Fellowship, Assistant Program Director, Internal Medicine Residency, Jefferson Medical College of Thomas Jefferson University

Srikanth Nagalla, MBBS, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Nothing to disclose.

Acknowledgements

Wadie F Bahou, MD Chief, Division of Hematology, Hematology/Oncology Fellowship Director, Professor, Department of Internal Medicine, State University of New York at Stony Brook

Wadie F Bahou, MD is a member of the following medical societies: American Society of Hematology

Disclosure: Nothing to disclose.

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Differential diagnosis of thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome.
 
 
 
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