Síndrome urémico hemolítico atípico

¿Una entidad subdiagnosticada? Características clínicas y analíticas a propósito de dos casos

  • Ricardo Silvariño Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Centro de Nefrología. Sanatorio Americano, Departamento de Urgencia y Medicina Interna.
  • Gerardo Pérez Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Centro de Nefrología.
  • Orlando Canzani Cooperativa Médica de Florida (COMEF)
  • Patricia Larre Borges Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Centro de Nefrología.
  • Nelson Acosta Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Centro de Nefrología.
  • Óscar Noboa Universidad de la República, Facultad de Medicina, Hospital de Clínicas, Centro de Nefrología.
Palabras clave: SÍNDROME HEMOLÍTICO-URÉMICO, MICROANGIOPATÍAS TROMBÓTICAS, INTERCAMBIO PLASMÁTICO

Resumen

La microangiopatía trombótica (MAT) es una condición anátomo-patológica caracterizada por lesión de la pared vascular con engrosamiento parietal, edema y desprendimiento de células endoteliales de la membrana basal. Clínicamente se presenta como anemia hemolítica microangiopática, oclusión microvascular por trombos plaquetarios y trombocitopenia. Puede ser consecuencia de una alteración primaria en el complejo sistema que regula la relación entre endotelio y coagulación u ocurrir en el contexto de una alteración sistémica. Dos fenotipos de la enfermedad son el púrpura trombocitopénico trombótico (PTT) y el síndrome urémico hemolítico (SUH). La disponibilidad de herramientas terapéuticas hace necesario un rápido reconocimiento de este mecanismo patogénico para poner en marcha una terapéutica precoz. Se presentan dos casos de SUH atípico y a partir de ellos se revisan los principales aspectos diagnósticos, terapéuticos y pronósticos de la enfermedad.

Citas

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Publicado
2014-03-31
Cómo citar
1.
Silvariño R, Pérez G, Canzani O, Larre Borges P, Acosta N, Noboa Óscar. Síndrome urémico hemolítico atípico. Rev. Méd. Urug. [Internet]. 31 de marzo de 2014 [citado 19 de mayo de 2022];30(1):56-4. Disponible en: http://revista.rmu.org.uy/ojsrmu311/index.php/rmu/article/view/260

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