Síndrome urémico hemolítico atípico: una complicación postoperatoria infrecuente

espanolIntroduccion y objetivos La purpura trombocitopenica trombotica y el sindrome uremico hemolitico atipico (SHUa) son microangiopatias tromboticas agudas, infrecuentes y potencialmente fatales que requieren una gestion rapida. Reportamos un caso de anemia hemolitica microangiopatica aguda (MAHA) que se presento en el entorno perioperatorio. Caso clinico Tras la realizacion de periquistectomia hepatica debida a quiste hidatidico, una mujer de 46 anos desarrollo MAHA, trombocitopenia e insuficiencia renal aguda durante el periodo postoperatorio inmediato. Se considero la posibilidad de SHUa, iniciandose intercambio de plasma inmediato. Se realizo intercambio de plasma durante 2 semanas, con remision de la disfuncion renal. Posteriormente se evaluaron las mutaciones geneticas y las causas inmunologicas de MAHA. Se identificaron mutaciones en el factor H del complemento asociadas a deficiencia del factor H, que estan asociadas a un incremento del riesgo de SHUa. Conclusion MAHA constituye una situacion postoperatoria infrecuente, que requiere un rapido diagnostico diferencial y tratamiento. Los anestesiologos deberan considerar el SHUa como causa posible de MAHA, especialmente en lo referente al cuidado inmediato de estos pacientes. EnglishIntroduction and objectives Thrombotic thrombocytopenic purpura and atypical haemolytic uremic syndrome (aHUS) are acute, rare, life-threatening thrombotic microangiopathies that require swift management. We report a case of acute microangiopathic haemolytic anaemia (MAHA) presenting in perioperative setting. Clinical case After hepatic pericystectomy for hydatid cyst, a 46-year-old female developed MAHA, thrombocytopenia and acute renal failure in the immediate postoperative period. “aHUS” was considered and immediate plasma exchange was initiated. Plasma exchange was performed for 2 weeks with remission of renal dysfunction. Further evaluation of genetic mutations and immunological causes for MAHA were sought. Mutations in complement factor H associated with factor H deficiency were identified, which are associated with increased risk of aHUS. Conclusion MAHA is a rare postoperative condition, requiring rapid differential diagnosis and treatment. Anaesthetists should bear in mind aHUS as a possible cause of MAHA, especially concerning immediate care for these patients.