Title: Pregnancy Associated Atypical Hemolytic Uremic Syndrome – A Two-Case Report of a Rare Entity
Authors: Tanvi Katoch, Mahak Bhardwaj, Aashima Arora
DOI: https://dx.doi.org/10.18535/jmscr/v11i4.11
Abstract
Atypical hemolytic uremic syndrome (aHUS) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. Atypical HUS is not caused by infections, rather have a genetic predisposition and pregnancy is one of the triggers. Being a rare entity and due to other masquerading events in pregnancy, it can be masked and lead to late diagnosis and increased morbidity. We present two cases of aHUS triggered by pregnancy, which were managed timely and discharged successfully, though long-term prognosis is poor. Recombinant monoclonal antibody therapy Eculizumab delays the progression of disease but is very expensive and most of the patients can’t afford it, as it is a long term therapy. Plasma exchange remains the mainstay of treatment initially and later on, if eculizumab is unaffordable. The main challenges are identification of the disease and timely intervention before increase in morbidity.
Keywords: atypical haemolytic uremic syndrome, pregnancy, plasma exchange, eculizumab.
References
- Bruel A, Kavanagh D, Noris M, et al. Hemolytic Uremic Syndrome in Pregnancy and Postpartum. Clin J Am Soc Nephrol. 2017 Aug 7;12(8):1237-1247. doi: 10.2215/CJN.00280117.
- Saad AF, Roman J, Wyble A, et al. Pregnancy-Associated Atypical Hemolytic-Uremic Syndrome. AJP Rep. 2016 Mar;6(1):e125-8. doi: 1055/s-0036-1579539
- Fakhouri F, Zuber J, Frémeaux-Bacchi V, et al. Haemolytic uraemic syndrome. The Lancet. 2017 Aug 12;390(10095):681-96. https://doi.org/10.1016/S0140-6736(17)30062-4
- Kavanagh D, Goodship T, Richards A, Atypical haemolytic uraemic syndrome, British Medical Bulletin. 2006; 77-78 (1): 5-22. https://doi.org/10.1093/bmb/ldl004
- Legendre CM, Licht C, Muus P, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med2013; 368: 2169–2181. doi: 10.1056/NEJMoa1208981
- Gaggl M, Aigner C, Csuka D, et al. Maternal and fetal outcomes of pregnancies in women with atypical hemolytic uremic syndrome. Journal of the American Society of Nephrology. 2018; 29(3): 1020–1029. doi: 10.1681/ASN.2016090995