Microangiopatías trombóticas: púrpura trombocitopénica trombótica y síndrome hemolítico urémico. Una revisión más allá del espectro clínico de la enfermedad
Resumen
La microangiopatía trombótica se relaciona con una lesión histológica en las arteriolas y los capilares; se caracteriza por engrosamiento e inflamación de la pared vascular, desprendimiento de células endoteliales, ampliación del espacio subendotelial por acumulación de proteínas, material de lisis celular y trombos plaquetarios que ocluyen la luz vascular. Hay dos enfermedades con lesiones de microangiopatía trombótica primaria: la púrpura trombocitopénica trombótica y el síndrome hemolítico urémico. La púrpura trombocitopénica trombótica se relaciona con un déficit o disfunción de la actividad de la desintegrina y la metaloproteasa, que escinden el enlace del factor de von Willebrand; una proteína que participa en el proceso de la coagulación. Desde el punto de vista clínico se caracteriza por una anemia hemolítica microangiopática, trombocitopenia y aumento de la deshidrogenasa láctica sérica. El síndrome hemolítico urémico se define por la anemia hemolítica microangiopática, trombocitopenia y la lesión renal aguda. Se clasifica en típico y atípico. El primero se asocia con el pródromo de diarrea posterior a una infección por Escherichia coli productora de toxina Shiga. Por su parte, el síndrome hemolítico urémico atípico se relaciona con anormalidades en la vía alternativa del complemento, infecciones por Streptococcus pneumoniae, lupus eritematoso sistémico, trastornos del metabolismo de la cobalamina C, entre otros.
Citas
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