Hemofilia A adquirida : reporte de un caso y revisión de la literatura.
Acquired haemophilia A : case report and literature review.
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La hemofilia adquirida A es una enfermedad autoinmune rara caracterizada por la presencia de autoanticuerpos contra el factor VIII de la coagulación; se manifiesta clínicamente como un síndrome hemorrágico, principalmente mucocutáneo, subcutáneo y en tejidos blandos. La etiología es idiopática en la mayoría de los casos; sin embargo, en los pacientes con patologías asociadas, las más frecuentes son enfermedades del colágeno, tumores sólidos, puerperio, tumores hematolinfoides y medicamentos. Está asociada con una morbilidad y mortalidad significativas, fundamentalmente en los pacientes de mayor edad. Describimos el caso de una mujer de 90 años, que debutó con sangrado subcutáneo severo asociado a prolongación del aPTT. En el abordaje diagnóstico, se encontró una actividad del factor VIII marcadamente disminuida y niveles de inhibidores contra el factor VIII elevados (120 BU/ml), sin antecedente previo de trastornos de la coagulación. Se inició tratamiento con corticoides y se adicionó posteriormente ciclofosfamida, logrando disminuir los títulos del inhibidor y acortar el aPTT; no obstante, la paciente falleció a las tres semanas debido a un ataque cerebro-vascular isquémico no relacionado con el tratamiento instaurado.
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