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Congenital afibrinogenemia - Treatment

Treatment:

To treat bleeding episodes or to prepare for surgery to treat other conditions, patients may receive:

  • Cryoprecipitate (a blood product containing concentrated fibrinogen and other clotting factors) through a vein (transfusion)
  • Fibrinogen (RiaSTAP)
  • Plasma (the liquid portion of the blood containing clotting factors)

People with this condition should have the hepatitis B vaccine because transfusion increases the risk of hepatitis.

Expectations (prognosis):

Excess bleeding is common with this condition. These episodes may be severe, or even fatal. Bleeding in the brain is a leading cause of death in patients with this disorder.

Complications:

  • Bleeding from the umbilical cord
  • Bleeding from the mucus membranes
  • Bleeding in the brain (intracranial bleeding)
  • Clotting with treatment
  • Development of antibodies (inhibitors) to fibrinogen with treatment
  • Gastrointestinal bleeding
  • Miscarriage

Calling your health care provider:

Call your health care provider or seek emergency care if you have excessive bleeding.

Tell your surgeon before you have surgery if you know or suspect you have a bleeding disorder.

  • 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.

References

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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