Thrombotic thrombocytopenic purpura in pregnancy: a comprehensive review

Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hemolytic disorder with severe implications during pregnancy, characterized by microangiopathic hemolytic anemia (MAHA), severe thrombocytopenia, and systemic microvascular thrombosis. The condition arises from a severe deficiency in the ADAMTS13 enzyme, whether congenital or acquired, leading to the accumulation of ultra-large von Willebrand factor (vWF) multimers and widespread platelet aggregation. Pregnancy itself exacerbates TTP due to physiological reductions in ADAMTS13 activity, necessitating a high degree of clinical vigilance. This review addresses the etiology, diagnostic challenges, clinical presentation, and management of TTP in pregnancy, focusing on clinical relevance and emphasizing the importance of prompt therapeutic plasma exchange (TPE) and interdisciplinary care to optimize maternal and fetal outcomes.