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 consent
form (ICF).

2. Clinical diagnosis of acquired TTP (initial or recurrent), which includes
thrombocytopenia and microscopic evidence of red blood cell fragmentation (e.g.,
schistocytes).

3. Requires initiation of daily PE treatment and has received 1 PE treatment prior to
randomization

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 and
received caplacizumab or for whom the assigned treatment arm is unknown

6. Others as defined in the protocol