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Deficiencia del factor V - Overview

Nombres alternativos

Parahemofilia; Enfermedad de Owren

Definición:

Es un trastorno hereditario que afecta la capacidad de la sangre para coagularse.

Causas, incidencia y factores de riesgo:

La coagulación sanguínea normal es un proceso complejo que involucra hasta 20 proteínas diferentes en el plasma sanguíneo, conocidas como factores de coagulación de la sangre. Con el uso de estos factores, tiene lugar rápidamente una serie de reacciones químicas complejas para formar una proteína llamada fibrina, la cual detiene el sangrado.

La deficiencia del factor V es causada por una falta del factor V de proteína en el plasma. Cuando faltan ciertos factores de la coagulación o son deficientes, la reacción en cadena no tiene lugar en forma normal. La deficiencia del factor V es poco común y puede ser causada al heredar un gen defectuoso del factor V o al adquirir un anticuerpo que interfiera con la función normal de este factor.

Usted puede adquirir un inhibidor del factor V:

  • Después de dar a luz
  • Después de ser tratado con un cierto tipo de goma de fibrina
  • Después de una cirugía
  • Con enfermedades autoinmunitarias y ciertos cánceres

Algunas veces se desconoce la causa.

Esta enfermedad es similar a la hemofilia, excepto que el sangrado intrarticular es menos común. En la forma hereditaria de deficiencia del factor V, un antecedente familiar de un trastorno hemorrágico es un factor de riesgo.

Frecuentemente, se presenta sangrado excesivo con los períodos menstruales y después del parto. Los hombres y las mujeres resultan afectados por igual. Alrededor de 1 persona por cada millón padece el trastorno.

  • Reviewed last on: 3/28/2010
  • David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Asselta R, Tenchini ML, Duga S. Inherited defects of coagulation Factor V: the hemorrhagic side. J Thromb Haemost. 2006 Jan;4(1):26-34.

Kessler CM. Hemorrhagic disorders: coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 180.

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