Atypical hemolytic uremic syndrome: Review of clinical presentation, diagnosis and management

Journal of Immunological Methods
Meera SridharanMaria A V Willrich

Abstract

Thrombotic microangiopathies (TMA) are a class of disorders characterized by microangiopathic hemolytic anemia, non-immune thrombocytopenia, and organ dysfunction. One type of TMA is atypical hemolytic uremic syndrome (aHUS) a disorder caused by hyper-activation of the alternative complement pathway due to over activation of C3 convertases and loss of complement regulatory mechanisms. The pathophysiological mechanism of aHUS involves increased continuous spontaneous hydrolysis of C3 to C3b which leads to tissue deposition of C3b, the membrane attack complex formation and subsequent tissue injury. The underlying susceptibility factors to aHUS include acquired autoantibodies or germline mutations in complement proteins or their regulators. Currently there are no clear diagnostic criteria for aHUS. Diagnosis involves ruling out other causes of TMA and incorporating complement serologic and genetic data. TPE has been used to treat aHUS; however, clinical improvement in these patents is far less than in patients with thrombotic thrombocytopenic purpura. Furthermore, there is a higher rate of progression to end stage renal disease with almost half of patients progressing despite TPE. For those, another option for treatment is eculizu...Continue Reading

Citations

Jun 30, 2019·European Journal of Haematology·Meera SridharanUNKNOWN Mayo Clinic Complement Alternative Pathway-Thrombotic Microangiopathy Disease-Oriented Group
Apr 28, 2020·The Journal of Maternal-fetal & Neonatal Medicine : the Official Journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians·Arthur J VaughtRobert A Brodsky
Nov 30, 2018·Frontiers in Immunology·A Inkeri LokkiHannele Laivuori
Mar 27, 2020·Clinical Epidemiology·Kevin YanChakrapani Balijepalli
May 9, 2021·Critical Reviews in Clinical Laboratory Sciences·Maria Alice V WillrichAshley Frazer-Abel
Jun 22, 2021·Frontiers in Medicine·Lara KollbrunnerAndreas W Jehle

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