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A combination regimen of **caplacizumab**, **corticosteroids**, and an **anti-CD20 antibody** (most commonly rituximab) is used for the management of **acquired thrombotic thrombocytopenic purpura (aTTP)**. Caplacizumab is a humanized bivalent Nanobody that targets the A1-domain of von Willebrand factor, thereby blocking platelet aggregation and preventing microvascular thrombosis. Corticosteroids act as immunosuppressive agents to dampen the autoimmune response. Anti-CD20 antibodies (such as rituximab or obinutuzumab) deplete B cells, reducing the formation of pathogenic autoantibodies against ADAMTS13. This combination approach addresses both immediate microthrombosis and underlying autoimmunity, leading to improved platelet recovery, lower relapse rates, and improved survival in aTTP patients[1][3][7][8][9].
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