Thrombotic thrombocytopenic purpura in pregnancy: Lessons from a case series of three patients

TL;DR

This paper discusses three cases of a rare blood disorder during pregnancy and highlights the importance of early diagnosis and tailored treatment to improve outcomes for mothers and babies.

Contribution

The paper presents three case studies highlighting treatment strategies and outcomes for thrombotic thrombocytopenic purpura during pregnancy.

Findings

Therapeutic plasma exchange remains critical for treating thrombotic thrombocytopenic purpura during pregnancy.

Caplacizumab effectively managed a severe relapsing case of thrombotic thrombocytopenic purpura during pregnancy.

Monitoring ADAMTS13 activity is essential for distinguishing congenital from acquired cases and guiding treatment.

Abstract

Thrombotic thrombocytopenic purpura is a rare but life-threatening complication during pregnancy. 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–von 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. These cases underscore the importance of early diagnosis, individualized treatment, multidisciplinary care, and proactive management to optimize maternal and neonatal outcomes.

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