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Monoclonal gammopathy of renal significance triggering atypical haemolytic uraemic syndrome
Author(s) -
Mahmood Usman,
Isbel Nicole,
Mollee Peter,
Mallett Andrew,
Govindarajulu Sridevi,
Francis Ross
Publication year - 2017
Publication title -
nephrology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.752
H-Index - 61
eISSN - 1440-1797
pISSN - 1320-5358
DOI - 10.1111/nep.12934
Subject(s) - thrombotic microangiopathy , medicine , eculizumab , atypical hemolytic uremic syndrome , monoclonal gammopathy of undetermined significance , plasmapheresis , renal function , gastroenterology , haemolysis , microangiopathic hemolytic anemia , incidence (geometry) , haemolytic uraemic syndrome , monoclonal , immunology , monoclonal antibody , antibody , platelet , complement system , thrombotic thrombocytopenic purpura , disease , biochemistry , physics , chemistry , escherichia coli , optics , gene
Haemolytic uraemic syndrome is a rare condition with an overall incidence of one to two cases in a population of 100 000 and approximately 10% of these cases are classified as atypical.[1][Noris M, 2009] Atypical haemolytic uraemic syndrome (aHUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and acute kidney injury. aHUS can be genetic, acquired or idiopathic (negative genetic screening and no environmental triggers). We describe a case of aHUS triggered by monoclonal gammopathy of renal significance (MGRS) successfully treated with plasmapheresis and a bortezomib‐based chemotherapy regimen, resulting in marked improvement in renal function and other markers of haemolysis. This patient has been in remission for more than 2 years currently.