Monoclonal gammopathy of renal significance triggering atypical haemolytic uraemic syndrome.
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.
Department of Nephrology, Princess Alexandra Hospital, Queensland, Australia.
Usman
U
Mahmood
Department of Nephrology, Princess Alexandra Hospital, Queensland, Australia.
Nicole
N
Isbel
Department of Haematology, Princess Alexandra Hospital, Queensland, Australia.
Peter
P
Mollee
Royal Brisbane and Women's Hospital, Queensland, Australia.
Andrew
A
Mallett
Toowoomba Base Hospital, Toowoomba, Queensland, Australia.
Sridevi
S
Govindarajulu
Department of Nephrology, Princess Alexandra Hospital, Queensland, Australia.
Ross
R
Francis
D058186:Acute Kidney Injury / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D065766:Atypical Hemolytic Uremic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D005260:Female; D006801:Humans; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000175:diagnosis / Q000628:therapy
Acute kidney injury; Atypical haemolytic uraemic syndrome; Bortezomib; Complement; Membranoproliferative glomerulonephritis; Monoclonal gammopathy of renal significance; Thromobotic microangiopathy