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Afibrinogenemia congénita - Treatment

Tratamiento:

Para tratar episodios de sangrado o prepararse para una cirugía con el fin de tratar otras afecciones, los pacientes pueden recibir:

  • Crioprecipitado (un hemoderivado que contenga fibrinógeno concentrado y otros factores de la coagulación), a través de una vena (transfusión).
  • Fibrinógeno (RiaSTAP).
  • Plasma (la porción líquida de la sangre que contiene factores de la coagulación).

Las personas con esta afección deben hacerse aplicar la vacuna contra la hepatitis B, ya que la transfusión incrementa el riesgo de hepatitis.

Pronóstico:

Es común que se presente un sangrado excesivo con esta afección y los episodios pueden ser severos e incluso mortales. El sangrado cerebral es una causa importante de muerte en pacientes con este trastorno.

Posibles complicaciones:

  • Sangrado del cordón umbilical.
  • Sangrado de las membranas mucosas.
  • Sangrado en el cerebro (sangrado intracraneal).
  • Coagulación con tratamiento.
  • Desarrollo de anticuerpos (inhibidores) para fibrinógeno con el tratamiento.
  • Sangrado gastrointestinal.
  • Aborto espontáneo.

Cuándo contactar a un profesional médico:

Consulte con el médico o busque atención médica de urgencia si tiene un sangrado excesivo.

Coméntele al cirujano antes de una cirugía si usted sabe o sospecha que sufre de un trastorno hemorrágico.

  • Reviewed last on: 2/28/2011
  • David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Referencias

Gailani D, Neff AT. Rare coagulation factor deficiencies. In: Hoffman R, Benz EJ Jr., Shattil SJ, et al, eds. Hoffman Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Churchill Livingstone Elsevier; 2008:chap 127.

Kessler C. 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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