Treating Immune Thrombotic Thrombocytopenic Purpura with Therapeutic Plasma Exchange

Therapeutic plasma exchange (TPE) has served as a first-line therapy for immune thrombotic thrombocytopenic purpura (iTTP) for decades, providing a rapid reduction in the causative anti-ADAMTS13 antibodies and replenishment of ADAMTS13 enzyme activity. Management of iTTP using TPE requires understanding the clinical and technical aspects of the procedure in order to provide safe and effective treatment. Furthermore, the apheresis practitioner must work within the context of a multidisciplinary team to individualize treatment to the patient and clinical circumstances. This chapter details the role of the apheresis consultant in assessing the patient, establishing treatment parameters, and managing treatment complications. The goal of the authors is to provide a blueprint for the stepwise process from differential diagnosis to disease resolution, including recommendations for developing a systematic treatment regimen, adjusting procedural settings to prevent and address complications, and modifying the treatment approach for unique patient populations.