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Treating Immune Thrombotic Thrombocytopenic Purpura with Immunosuppressive Agents
1Division of Hematologic Malignancies and Cellular Therapeutics, Department of Internal Medicine, The University of Kansas Medical Center, Kansas City, KS, USA. khalkidis@kumc.edu.
Immune thrombotic thrombocytopenic purpura (iTTP) is caused by autoimmune inhibition of the metalloprotease ADAMTS13. Despite recent advances in diagnosis and therapeutics, most patients are treated empirically with a combination of immunosuppression and therapeutic plasma exchange (TPE) while awaiting diagnostic confirmation of the disease. Once diagnosis is confirmed, immunosuppression is continued in both the short and intermediate term to prevent the ongoing acute sequelae of the disease as well as prevent relapse of iTTP. Here, I describe approaches for immunosuppressive therapy in patients with iTTP.
Immune thrombotic thrombocytopenic purpura (iTTP) is caused by autoimmune inhibition of the metalloprotease ADAMTS13. Despite recent advances in diagnosis and therapeutics, most patients are treated empirically with a combination of immunosuppression and therapeutic plasma exchange (TPE) while awaiting diagnostic confirmation of the disease. Once diagnosis is confirmed, immunosuppression is continued in both the short and intermediate term to prevent the ongoing acute sequelae of the disease as well as prevent relapse of iTTP. Here, I describe approaches for immunosuppressive therapy in patients with iTTP.
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