Monoclonal gammopathy of renal significance triggering atypical haemolytic uraemic syndrome

• Published in: Nephrology (Carlton, Vic.) Vol. 22; no. S1; pp. 15 - 17
• Main Authors: Mahmood, Usman, Isbel, Nicole, Mollee, Peter, Mallett, Andrew, Govindarajulu, Sridevi, Francis, Ross
• Format: Journal Article
• Language: English
• Published: Australia Wiley Subscription Services, Inc 01.02.2017
• Subjects: Acute kidney injury; Acute Kidney Injury - complications; Acute Kidney Injury - diagnosis; Acute Kidney Injury - therapy; Anemia; Atypical haemolytic uraemic syndrome; Atypical Hemolytic Uremic Syndrome - diagnosis; Atypical Hemolytic Uremic Syndrome - etiology; Atypical Hemolytic Uremic Syndrome - therapy; Bortezomib; Chemotherapy; Complement; Female; Genetic screening; Hemolytic anemia; Humans; Membranoproliferative glomerulonephritis; Middle Aged; Monoclonal gammopathy; Monoclonal gammopathy of renal significance; Paraproteinemias - complications; Paraproteinemias - diagnosis; Paraproteinemias - therapy; Plasmapheresis; Remission; Renal function; Thrombocytopenia; Thrombotic microangiopathy; Thromobotic microangiopathy; Thromobotic microangiopathy; Atypical haemolytic uraemic syndrome; Bortezomib; Membranoproliferative glomerulonephritis; Acute kidney injury; Monoclonal gammopathy of renal significance; Complement
• ISSN: 1320-5358; 1440-1797
• DOI: 10.1111/nep.12934

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. 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.