Monoclonal gammopathy of renal significance triggering atypical haemolytic uraemic syndrome

Mahmood, Usman;Isbel, Nicole;Mollee, Peter;Mallett, Andrew;Govindarajulu, Sridevi;Francis, Ross

Abstract

Haemolytic uraemic syndrome is a rare condition with an overall incidence ofone to two cases in a population of 100 000 and approximately 10% of thesecases are classiﬁed as atypical.1Atypical haemolytic uraemic syndrome (aHUS)is a thrombotic microangiopathy (TMA) characterized by microangiopathichaemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury.aHUS can be genetic, acquired or idiopathic (negative genetic screening andno environmental triggers). We describe a case of aHUS triggered by monoclo-nal gammopathy of renal signiﬁcance (MGRS) successfully treated with plas-mapheresis and a bortezomib-based chemotherapy regimen, resulting inmarked improvement in renal function and other markers of haemolysis. Thispatient has been in remission for more than 2 years currently.

Journal

Nephrology

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1440-1797

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Suppl. 1

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Publisher

Wiley-Blackwell Publishing Asia

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DOI

10.1111/nep.12934