Thrombotic Thrombocytopenic Purpura: Lessons from Clinical Cases, A case series

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Abstract

Thrombotic Thrombocytopenic Purpura (TTP) is a rare yet life-threatening complication during pregnancy, with maternal mortality rates historically exceeding 90% before the advent of therapeutic plasma exchange (TPE), which has been the cornerstone of treatment for several decades. Rituximab has become increasingly important for managing refractory or relapsing TTP, particularly during pregnancy, due to its effectiveness in reducing relapses. In severe or recurrent cases, caplacizumab has emerged as a valuable agent, rapidly controlling acute episodes by preventing platelet aggregation and reducing microvascular thrombosis, which is critical in reducing organ damage and improving survival. Monitoring ADAMTS13 activity has become crucial in distinguishing between congenital and acquired TTP, guiding treatment decisions, and preventing relapses. This case series includes both acquired and congenital TTP cases, and in one severe case with multiple relapses, we used caplacizumab to stabilize the patient's condition. The findings underscore the importance of early diagnosis, individualized treatment, and proactive management in improving maternal and neonatal outcomes.

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