Abstract

LUQUIN-IRIGOYEN, M; ARMENDARIZ-BRUGOS, C  and  VALLEJO-RUIZ, M. Diagnostic clues in atypical hemolytic uremic syndrome: a case report. Anales Sis San Navarra [online]. 2022, vol.45, n.2, e1006.  Epub Apr 24, 2023. ISSN 1137-6627.  https://dx.doi.org/10.23938/assn.1006.

Atypical hemolytic uremic syndrome (aHUS) is a clinical entity characterized by non-immune hemolytic anemia, thrombocytopenia and renal failure, in which lesions are mediated by a systemic thrombotic microangiopathy. It is a rare pathology whose origin is a complement system deregulation due to mutations in its genes that lead to uncontrolled activation of C5 and the formation of the membrane attack complex.- Its correct diagnosis allows us to prescribe the treatment based on Eculizumab, a C5 inhibitor.

We report the case of a pregnant patient with aHUS, with the aim of highlighting the importance of early differential diagnosis to establish an early and effective treatment of this pathology. The pathophysiology, diagnosis and genetic study are updated, as well as the therapeutic management of aHUS.

Keywords : Atypical hemolytic-uremic síndrome; Microangiopathic hemolytic anemia; Complement pathway alternative; Complement System Proteins; Eculizumab.

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