Clinical Efficacy and Safety Profile of Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is usually defined as microangiopathy characterized by low platelet count and low red blood cell count, i.e., hemolytic anemia. It can either be acquired or immune-mediated. TTP requires quick diagnostic identification and emergent management. According to the evidence-based guidelines, the recommended therapy is plasma exchange and immunosuppression. Caplacizumab is used alongside the standard recommended therapy. Caplacizumab is a monoclonal antibody (Mab) that binds to von Willebrand factor (VWF). This prevents A1 VWF to bind platelet glycoprotein 1b receptor. The recommended dosage for this drug is 10mg. At the start, 10mg intravenous (IV) dose is given before plasma exchange, followed by daily 10mg subcutaneous (SC) dose after plasma exchange. Moreover, the SC dose is continued even after the daily plasma exchange is stopped. This review aims to consolidate findings related to the efficacy of this recently approved drug.