ATYPICAL HAEMOLYTIC URAEMIC SYNDROME IN AUSTRALIA: REAL-WORLD CHALLENGES AND LESSONS FROM THE THROMBOTIC MICROANGIOPATHY REGISTRY

KJ ROBSON1, NM ISBEL2, TD BARBOUR3, EM WOOD4, JY KAUSMAN5,6,T DE MALMANCHE7,8, PA BLOMBERY9, PD HUGHES3, L KIVIVALI4, Z MCQUILTEN4, SJ COHNEY1

1Western Health, Melbourne; 2University of Queensland at Princess Alexandra Hospital, Brisbane; 3Royal Melbourne Hospital, Melbourne; 4Department of Epidemiology & Preventive Medicine, Monash University, Melbourne; 5Royal Children’s Hospital, Melbourne; 6Murdoch Research Institute, RCH, Melbourne; 7School of Medicine & Public Health, University of Newcastle, Newcastle; 8Pathology North (Hunter) Immunology, Newcastle; 9Peter MacCallum Cancer Centre, Melbourne

Aims: To characterize clinical presentations, genetic variants, treatment, and outcomes in Australian patients with aHUS. To highlight current diagnostic and therapeutic practices, and to identify potential areas for improvement
and research.

Background: Registry data is critical in furthering our understanding of aHUS, a rare, life-threatening disease. This is the first report on patients with aHUS from the Australia & New Zealand TMA registry (established 2009).

Methods: Registry data from June 2009 to January 2016 were reviewed. Cases registered as ‘aHUS’ were correlated with the original medical record by a single investigator. Data were analysed with descriptive statistics, and subgroups compared using Mann-Whitney U test.

Results: 38 patients with aHUS were included: 32 adults, 6 children. 6/38 presented after solid-organ transplant, and 5/38 after exposure to a drug associated with TMA. ADAMTS13 activity was >10% in the 24/38 cases where it was measured. Thrombocytopenia and renal impairment were less severe in post-transplant and drug-associated cases compared with the remainder of the cohort. 18/38 (47%) patients underwent dialysis; 2 remained dialysis-dependent at follow-up, 4 underwent subsequent renal transplantation. Complement gene analysis was performed in 14 cases, with 10 risk variants identified in 9 patients. Plasma exchange (PEx) was provided in 30/38 (79%) cases. PEx duration was significantly longer in cases without measured ADAMTS13 activity (median 15 vs. 4.5 days, p=0.04). 23 patients received eculizumab; 16/23 were treated during first admission.

Conclusions: Characteristics of Australian patients with aHUS were similar to other reported cohorts, but subgroup analysis revealed significant differences between post-transplant, drug-associated, and other cases. This study identified practical challenges in diagnostic workup and treatment of aHUS in Australia, highlighting areas for potential improvement in patient care.

About ANZSN

The ASM is hosted by Australian and New Zealand Society of Nephrology.

The aims of the Society are to promote and support the study of the kidney and urinary tract in health and disease, and to ensure the highest professional standards for the practice of nephrology in Australia and New Zealand.

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