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Historically, maternal mortality exceeded 90% before the introduction of therapeutic plasma exchange, which remains the cornerstone of treatment. Rituximab has become increasingly valuable in managing refractory or relapsing disease, even during pregnancy, by reducing the risk of future episodes. In severe or recurrent cases, caplacizumab provides rapid control of acute thrombotic thrombocytopenic purpura by inhibiting platelet\u2013von Willebrand factor interaction, although its use in pregnancy remains limited. Monitoring ADAMTS13 activity is essential to distinguish congenital from acquired thrombotic thrombocytopenic purpura, guide treatment decisions, and prevent relapses. This case series describes one acquired and two congenital thrombotic thrombocytopenic purpura cases in pregnancy, including a severe relapsing case successfully treated with caplacizumab. 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