Introduction
ischemia, hemolytic anemia, TTP, Thrombotic thrombocytopenia purpura
HEMOLYTIC ANEMIA, THROMBOCYTOPENIA, ISCHEMIA, ITTP, TTP
Thrombotic thrombocytopenia purpura (TTP) is an acute life-threatening condition characterized by thrombocytopenia, non-immune hemolytic anemia and organ ischemia [The acquired, or immune-mediated, form of TTP (iTTP) is rare, but more prevalent than the hereditary form [Approximately 18% of individuals with iTTP are refractory to the standard treatments [Caplacizumab is a humanized, single-chain dimer, bivalent, variable-domain-only immunoglobulin fragment directed against the A1 domain of VWF, which inhibits the interaction between VWF and glycoprotein Ib-IX-V receptors on platelets [Thus, the aim of the current phase 2/3 study was to evaluate the efficacy and safety of caplacizumab, in addition to standard care, in Japanese individuals with iTTP.