Adjuvant Low Dose Rituximab for Acquired TTP With Severe ADAMTS13 Deficiency
Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by small blood clots throughout the body that can damage major organs and cause death. TTP is treated with plasma exchange (also called plasmapheresis). Patients who do not respond initially to plasma exchange often are helped by later treatment with rituximab. The purpose of this study is to see whether combining low doses of rituximab with plasma exchange will help patients get better sooner and reduce the chance of getting TTP again.
• Age 18 or greater
• Diagnosis of suspected thrombotic thrombocytopenic purpura (TTP)
• Platelet count of < 80,000 for newly diagnosed patients and < 120,000 for relapsed patients
• Microangiopathic hemolytic anemia with RBC fragmentation
• LDH >1 x ULN
• Subjects who will receive treatment for TTP with plasma exchange
• Subjects who have not started the 5th plasma exchange
• Plasma ADAMTS13 activity <10%