TTP, is a rare blood disorder characterized by microscopic clots throughout blood vessels in the body. TTP can cause catastrophic damage if not quickly diagnosed and treated because sufficient oxygen is unable to reach vital organs. Patients are typically treated with plasma exchange therapy.
In an editorial published in the journal Transfusion, Drs. Joseph Kiss and Lynne Uhl review recent research on the use of immunomodulation therapy to help manage TTP.
Dr. Kiss, of the Institute for Transfusion Medicine and the University of Pittsburgh, explains:
“Several recent publications highlight the emerging role of immunosuppressive therapy particularly using the drug, rituximab in the management of patients with Thrombotic Thrombocytopenic Purpura or TTP. These studies have largely come from registries type data and they say the same thing which is that the use of the drug earlier in the management of TTP appears to shorten the period of thrombocytopenia and accelerate the time to the attainment of remission when treating this disorder by plasma exchange.”
In a study utilizing the Oklahoma registry, researchers evaluated the complication rate of TTP patients undergoing plasma exchange. They found a decreasing incidence of catheter-related infections over time. The researchers hypothesize that this reduction was attributable to fewer plasma exchange procedures resulting from increased use of corticosteroids and rituximab.
This study raised important questions about the potential use of rituximab and other immunosuppression therapies for TTP patients.
Prospective studies were also recently conducted to compare relapse rates among patients given early rituximab. In the United Kingdom, 40 TTP patients were given rituximab within three days of the first plasma exchange, as well as high doses of corticosteroids. These patients had significantly lower relapse rates, shorter periods of thrombocytopenia and shorter hospital stays compared to historical controls.
In addition, there have been several studies and case reports of the use of rituximab to maintain remission.
These data show rituximab and corticosteroids are useful at initial presentation of TTP and may have a role for relapsing patients. However, immune suppression treatments should be tailored to specific patients.
We’ll be back on January 15th with another edition of Transfusion News. In the meantime, you can always keep up to date with all of the latest news by visiting transfusionnews.com. Thanks for joining us.
- Kiss JE, Uhl L: Telltale signs of progress in the management of thrombotic thrombocytopenic purpura. Transfusion 2012;52:2498-2501.
- National Heart Lung and Blood Institute. National Insitutes of Health. Health Information for the Public. What Is Thrombotic Thrombocytopenic Purpura? http://www.nhlbi.nih.gov/health/health-topics/topics/ttp/ Accessed November 2, 2012.
- Som S, Deford CC, Kaiser ML, Terrell DR, Kremer Hovinga JA, Lammle B, George JN, Vesely SK: Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura-hemolytic uremic syndrome, 1996 to 2011. Transfusion 2012;52:2525-2532; quiz 2524.
- Bhagirath VC, Kelton JG, Moore J, Arnold DM: Rituximab maintenance for relapsed refractory thrombotic thrombocytopenic purpura. Transfusion 2012;52:2517-2523.
- Scully M, McDonald V, Cavenagh J, Hunt BJ, Longair I, Cohen H, Machin SJ. A Phase 2 Study of the Safety and Efficacy of Rituximab with Plasma Exchange in Acute Acquired Thrombotic Thrombocytopenic Purpura. Blood 2011 Aug 18;118(7):1746-53.