A life-threatening case of pregnancy-related atypical Haemolytic uremic syndrome and successful treatment with Eculizuma
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CASE REPORT
Open Access
A life-threatening case of pregnancyrelated atypical Haemolytic uremic syndrome and successful treatment with Eculizumab Prianka Puri1,2* , Anida Hanxhiu1, Daniel V. O’Hara1,3, Danny Hsu4 and Mirna Vucak-Dzumhur1,5
Abstract Background: Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS) is a rare condition affecting genetically predisposed women during pregnancy. It is often difficult to diagnose and has a significant impact on maternal and foetal outcomes. It is characterised by microangiopathic haemolytic anaemia and kidney injury from thrombotic microangiopathy. Case presentation: A 27-year-old female of Lebanese descent presented at 36 weeks’ gestation with foetal death in-utero (FDIU) with placental abruption on a background of previously normal antenatal visits. She was coagulopathic and anaemic with anuric acute kidney injury, requiring emergency Caesarean section, intubation and dialysis. Her coagulopathy rapidly resolved, however, her anaemia and renal dysfunction persisted. A diagnosis of P-aHUS was made, and she was empirically treated with Eculizumab. Her ADAMTS13 level was normal, effectively excluding thrombotic thrombocytopenic purpura. Within 2 weeks of treatment her haematological parameters improved, and her renal function began to recover and within 2 months she became dialysis independent. Conclusion: This case highlights the challenges of a timely diagnosis of P-aHUS from other pregnancy-related diseases. Although our patient is dialysis-independent, her risk of relapse remains high with subsequent pregnancies. Currently we are awaiting her genetic sequencing to complete her assessment for underlying mutations and are determining the safest approach to a future planned pregnancy. Keywords: Pregnancy-related atypical Haemolytic uremic syndrome, Eculizumab, Microangiopathic haemolytic anaemia, Thrombotic microangiopathy
Background During pregnancy, there are constant adaptations within the maternal system to promote the development of the foetus [1]. Part of these adaptations occur at the maternal-foetal interface, whereby there is an increased expression of complement regulator proteins. At the * Correspondence: [email protected] 1 The Westmead Hospital Nephrology and Transplant Unit, Westmead, NSW, Australia 2 The Canberra Hospital Nephrology Unit Cabrera ACT, Canberra, Australia Full list of author information is available at the end of the article
time of delivery, these regulator proteins may be diminished, and an exaggerated maternal response may trigger the excessive activation of the complement system and the development of Pregnancy-related Atypical Haemolytic Uremic Syndrome (P-aHUS). P-aHUS is thought to occur in genetically predisposed individuals who harbour mutations in complement regulator proteins or develop autoantibodies to complement factors. These mutations occur in the alternative complement pathway, most commonly in complement factor H, Complement factor
© The Author(s). 2020 Open Access This article is licensed under a Creative Co
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