AN ATYPICAL CASE: POST-PARTUM AHUS

S SO1,2, E FISCHER1, B BOSE1,3
1Department of Renal Medicine, Nepean Hospital, Kingswood, Australia, 2Department of Renal Medicine, Westmead Hospital, Westmead, Australia, 3University of Sydney, Nepean Clinical School, Kingswood, Australia

Background: Hypertensive complications of pregnancy, such as pre-eclampsia, are the leading cause of AKI in pregnancy globally. However, features of these syndromes can overlap with thrombotic microangiopathies (TMAs). Delayed treatment of TMAs correlate with worse renal outcomes
Case Report: A 27-year-old primigravida presented at 36 weeks gestation, with abdominal discomfort and hypertension. This is on a background of spina bifida with associated paraplegia. On Day 2, she had an emergency caesarean section for obstructed labour, complicated by postpartum haemorrhage, requiring blood transfusion. Coagulation profile was unremarkable.Immediately post-operatively, she developed oliguric acute kidney injury and haemolysis as demonstrated by reduced haptoglobin, elevated lactate dehydrogenase (LDH), fragmented red cells and raised reticulocyte count. She also had deranged liver function tests (LFTs). It was thought her presentation was due to severe pre-eclampsia/HELLP syndrome.She was commenced on dialysis. Although LFTs normalised within days, she remained dialysis-dependent with ongoing haemolysis. Vasculitic and autoimmune screen was unremarkable and direct antiglobulin test was negative. ADAMTS13 activity was normal. Ongoing haemolysis and renal impairment was considered more consistent with aHUS. She commenced Eculizumab on Day 10. By Day 28, she had ceased haemodialysis and haemolytic markers had normalised. Two years post-discharge, her renal function stabilised with a creatinine of 95 umol/L and there is no evidence of haemolysis.As she did not have risk factors for relapse and negative genetic testing for aHUS mutations, eculizumab was ceased after two years, without any evidence of relapse thus far.
Conclusions: Due to clinical overlap, hypertensive complications of pregnancy can be difficult to differentiate from TMAs. This differential should not be overlooked in patients who have ongoing haemolysis or end-organ dysfunction post-partum.


Biography:
Dr So is a second-year renal advanced trainee in the Western Sydney renal network.

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