Síndrome urémico hemolítico atípico. Reporte de caso y revisión de la bibliografía

INTRODUCCION: el sindrome uremico hemolitico, en su variedad atipica, es una microangiopatia trombotica poco frecuente, con elevadas morbilidad y mortalidad si no se establece el diagnostico oportuno que permita el tratamiento especifico adecuado. CASO CLINICO: paciente con diagnostico confirmado de sindrome uremico hemolitico atipico tratado con eculizumab con remision total y evolucion clinica satisfactoria. La sospecha diagnostica es importante para que el tratamiento sea temprano y especifico y el pronostico favorable. CONCLUSION: el caso aqui reportado es ilustrativo de la presentacion clinica del sindrome hemolitico uremico atipico. Su evolucion fue torpida, a pesar del tratamiento con infusion de plasma y plasmaferesis y su remision total con eculizumab. En Mexico se han confirmado pocos casos, solo en algunos se ha administrado el tratamiento especifico. PALABRAS CLAVE: sindrome uremico hemolitico atipico, microangiopatia trombotica, eculizumab Abstract INTRODUCTION: The hemolytic uremic syndrome in its atypical variety is a rare thrombotic microangiopathy, with high morbidity and mortality if a timely diagnosis is not available to allow an adequate specific treatment. CLINICAL CASE: Patient with a confirmed diagnosis of atypical hemolytic uremic syndrome treated with eculizumab with total remission and satisfactory clinical evolution. Diagnostic suspicion is important for early and specific treatment and favorable prognosis. CONCLUSION: The case reported here is illustrative of the clinical presentation of the atypical haemolytic uraemic syndrome. Its evolution was torpid, despite the treatment with plasma infusion and plasmapheresis and its total remission with eculizumab. In Mexico, few cases have been confirmed, only in some cases has the specific treatment been administered. KEYWORDS: atypical hemolytic uremic syndrome; thrombotic microangiopathy; eculizumab