Severe congenital thrombotic thrombocytopenic purpura in pregnancy complicated by eclampsia

A postpartum woman was diagnosed with congenital thrombotic thrombocytopenic purpura (TTP), triggered by pregnancy. She presented with severe thrombocytopenia, haemolysis, hypertension and neurological symptoms. Laboratory findings, including microangiopathic haemolytic anaemia, elevated lactate dehydrogenase (LDH) and a severely reduced <i>ADAMTS13</i> activity, confirmed the diagnosis. Genetic testing identified a mutation in the <i>ADAMTS13</i> gene. The patient was managed with plasma exchange (PLEX), fresh frozen plasma (FFP) and supportive care. After two sessions of PLEX, her platelet count increased to 150 × 10⁹/L, LDH normalised, blood pressure stabilised and antihypertensive medications were discontinued. At discharge, she was asymptomatic, with a creatinine of 0.6 mg/dL. Follow-up at 3 and 6 months showed no recurrence. This case highlights the importance of early recognition of TTP in pregnancy, differentiation from other pregnancy-related disorders and the role of genetic testing in guiding long-term management and counselling.