Abstract
BackgroundAtypical hemolytic uremic syndrome is an exceedingly rare thrombotic microangiopathy caused by accelerated activation of the alternative complement pathway.Case presentationHere, we report two cases of patients presenting with suspected atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 infection. The first patient, a 25-year-old Hispanic male, had one prior episode of thrombotic microangiopathy presumed to be atypical hemolytic uremic syndrome precipitated by influenza A, and re-presented with thrombocytopenia, microangiopathic hemolytic anemia, nonoliguric renal failure, and normal ADAMTS13 activity, with confirmed coronavirus disease 2019 positivity. The second patient, a 31-year-old Caucasian female, had no personal history of thrombotic microangiopathy, though reported a family history of suspected atypical hemolytic uremic syndrome. She presented with similar laboratory derangements, oliguric renal failure requiring hemodialysis, and confirmed coronavirus disease 2019 positivity. Both patients were treated with eculizumab with complete resolution of their hematologic and renal complications.ConclusionTo our knowledge, this represents the largest case series of atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 in adults.
Highlights
Atypical hemolytic uremic syndrome is an exceedingly rare thrombotic microangiopathy caused by accelerated activation of the alternative complement pathway.Case presentation: Here, we report two cases of patients presenting with suspected atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 infection
To our knowledge, this represents the largest case series of atypical hemolytic uremic syndrome precipitated by coronavirus disease 2019 in adults
Given the clinical picture including COVID19, depressed C3 complement level, acute kidney injury, normal ADAMTS13 level, and response to eculizumab, atypical Hemolytic uremic syndrome (HUS) (aHUS) triggered by COVID-19 was the most likely diagnosis
Summary
Atypical HUS is a rare diagnosis with high morbidity and a significant number of patients needing dialysis, mainly due to a delay in diagnosis. Diagnosis and initiation of treatment with eculizumab is of utmost importance and can help reduce the need for dialysis, permanent renal injury, and even death. COVID-19-triggered aHUS should be kept in differential and early institution of eculizumab therapy should be considered to improve patient outcomes
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