Phase III Trial With Caplacizumab in Patients With Acquired Thrombotic Thrombocytopenic Purpura

Inclusion Criteria:

1. Adult male or female ≥ 18 years of age at the time of signing the informed consentform (ICF).
2. Clinical diagnosis of acquired thrombotic thrombocytopenic purpura (aTTP) (initial orrecurrent), which included thrombocytopenia and microscopic evidence of red blood cellfragmentation (e.g., schistocytes).
3. Required initiation of daily PE treatment and had received 1 PE treatment prior torandomization
4. Others as defined in the protocol

Exclusion Criteria:

1. Platelet count ≥100×10E9/L.
2. Serum creatinine level >200 µmol/L in case platelet count is > 30×10E9/L
3. Known other causes of thrombocytopenia
4. Congenital TTP (known at the time of study entry).
5. Pregnancy or breast-feeding.
6. Subjects who were previously enrolled in a clinical study with caplacizumab andreceived caplacizumab or for whom the assigned treatment arm is unknown
7. Others as defined in the protocol