An Atypical Case of Atypical Hemolytic Uremic Syndrome: Predominant Gastrointestinal Involvement, Intact Renal Function, and C5b-9 Deposition in Colon and Skin

Jocelyn De Yao, Robert Kaplan, Cynthia Magro

Abstract


Atypical hemolytic uremic syndrome (aHUS) is one of the prototypic thrombotic microangiopathies which arises from a genetically-based defect in the regulatory control of the alternate complement cascade. Although its cause is distinct, it shares a similar clinical presentation with thrombotic thrombocytopenic purpura with respect to its pattern of organ involvement, with most cases including the renal, central nervous, and gastrointestinal systems. Renal dysfunction in aHUS is generally recognized as a clinical hallmark of the disease. We report a unique case of aHUS in a 71-year-old female highlighted by predominant effect on the gastrointestinal system concurrent with preservation of renal function and histopathologically-proven involvement of skin.




J Hematol. 2015;4(3):193-195
doi: http://dx.doi.org/10.14740/jh205w


Keywords


aHUS; Thrombotic microangiopathy

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