ARTICLE: Atypical HUS may become a diagnosis of inclusion
AUTHORS: J. Evan Sadler
JOURNAL: Blood. 2015 Jun 4;125(23):3525-6. doi: 10.1182/blood-2015-04-640656
Patients with aHUS usually have microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. However, these features occur in many diseases, including thrombotic thrombocytopenic purpura (TTP), and the more common form of Shiga toxin–expressing Escherichia coli hemolytic uremic syndrome. Making the correct diagnosis is critical because these disorders require different treatment. For example, treatment of aHUS with plasma exchange is associated with up to 8% mortality during the first episode and progression to end-stage renal failure in most survivors. However, treatment with the complement inhibitor eculizumab can halt the thrombotic microangiopathy, prevent or reverse renal failure, and forestall damage to the brain, heart, and other organs,2 which makes sense because aHUS is caused by defects in the regulation of the alternative complement pathway.
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