Abstract

SILVARINO, Ricardo et al. Síndrome urémico hemolítico atípico: ¿una entidad subdiagnosticada? Características clínicas y analíticas a propósito de dos casos. Rev. Méd. Urug. [online]. 2014, vol.30, n.1, pp.56-64. ISSN 1688-0390.

Abstract Thrombotic microangiopathy (MAT) is an anatomo-pathological condition characterized by a lesion in the arterial wall with parietal thickening, edema and endothelial cells detached from the basement membrane. Clinically it is seen as a hemolytic microangiopathic anaemia, microvascular occlusion due to platelete count and thrombocytopenia. It could be a consequence of a primary alteration in the complex system that regulates the relationship between the endothelio and coagulation or occur within the context of a systemic alteration. Thrombotic thrombocytopenic purpura (PPT) and hemolytic uremic syndrome (HUS) are two phenotypes of the disease. Availability of therapeutic tools calls for the fast acknowledgement of this pathogenic mechanism to start up early therapeutic. The study presents two cases of atypical HUS and based on that reviews the main diagnostic, therapeutic and prognostic aspects.

Keywords : HEMOLYTIC-UREMIC SYNDROME; THROMBOTIC MICROANGIOPATHIES; PLASMA EXCHANGE.

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