Background: Pregnancy-associated atypical haemolytic uraemic syndrome (p-aHUS) is a rare thrombotic microangiopathy (TMA) affecting one in 25,000 pregnancies. The most common disorders with features of TMA in pregnancy and postpartum are preeclampsia (PE) and HELLP syndrome. Other possible causes included thrombotic thrombocytopenic purpura (TTP) and p-aHUS. Differentiating PE, HELLP syndrome, TTP and p-aHUS is critical because the first-line treatment for p-aHUS is complement blockade with eculizumab. Corticosteroids and/or plasmapheresis are often attempted as first-line agents. These treatments are less effective for p-aHUS, and their use may increase maternal risk by delaying appropriate care.

Aims: We present a case of the 39-year old woman who was admitted to the hospital in the 37th week of pregnancy with gestational hypertension and foetal growth restriction. Her pregnancy was terminated by acute caesarean section due to imminent foetal hypoxia and placental abruption. Early after caesarean section, she developed acute respiratory distress syndrome, severe impairment of consciousness, anuric acute kidney injury, thrombocytopenia and microangiopathic haemolytic anaemia. Due to severe hemodynamic instability, eculizumab treatment and comprehensive supportive care, including continuous haemodialysis, were initiated immediately. Unfortunately, despite early treatment, she developed an end-stage renal disease. Additional genetic testing revealed inherited abnormalities in complement proteins – decreased expression of membrane cofactor protein (MCP, including in our case MCPggaac risk haplotype) and combination of variants within risk haplotype CFH-H3. Application of complement inhibitors continued, and 22 months later she underwent kidney transplantation.

Methods: not applicable

Results: not applicable

Conclusion(s): Our case presents the importance of interdisciplinary cooperation in pregnancy-associated TMA. Early administration of eculizumab probably saved our critically ill patient, and maintenance treatment contributed to the success of kidney transplantation.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/early-use-of-eculizumab-in-patient-with-pregnancy-associated-atypical-haemolytic-uraemic-syndrome/