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A.D.A.M. Medical Encyclopedia [Internet]. Atlanta (GA): A.D.A.M.; 2013.
A.D.A.M. Medical Encyclopedia.
Hemophilia refers to a group of bleeding disorders in which blooding takes a long time.
Some types of disorder include:
- Hemophilia A
- Hemophilia B
- Von Willebrand disease
Causes, incidence, and risk factors
When you bleed, the body sends out a special protein to help the blood clot. There is a higher risk of bleeding when one or more of these clotting factors are missing.
Hemophilia is caused by a lack of clotting factor VIII or IX in the blood. In most cases, hemophilia is passed down through families (inherited). Most of the time, it is passed to male children.
Symptoms
The main symptom of hemophilia is bleeding. Mild cases may not be detected until until later in life, after excessive bleeding following surgery or an injury.
In the worst cases, bleeding occurs for no reason. Internal bleeding may occur anywhere and bleeding into joints is common.
Signs and tests
Most often, hemophilia is diagnosed after a person has an abnormal bleeding episode Tests can also be done to detect the problem when other family members have the condition.
Treatment
The most common treatment is to replace the missing clotting factor in the blood through a vein (intravenous infusions).
You will need extra care during surgery if you have a bleeding disorder. Therefore, it is important to get a diagnosis. It will also help you to warn family members who might be affected.
Support Groups
Joining a support group where members share common issues can relieve the stress of a chronic disease.
Expectations (prognosis)
Many people with hemophilia are able to do most normal activities. However, some people have major bleeding events, The most common of these is bleeding into the spaces around the joints.
A small number of people with hemophilia may die from severe bleeding.
References
- Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 180.
- Ragni MV, Kessler CM, Lozier JN. Clinical aspects and therapy for hemophilia. 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 2005.
Review Date: 3/3/2013.
Reviewed by: Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.
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Recombinant factor VIIa for the prevention and treatment of bleeding in patients without haemophilia
The purpose of this review was to evaluate the evidence of effectiveness and safety for the use of recombinant factor VIIa (rFVIIa). This drug has been used in patients who are either at risk of major bleeding (e.g. because of planned high‐risk surgery), or who have uncontrolled bleeding (e.g. related to trauma). There have been many articles in the literature describing the off‐license use of this drug, which often suggest benefit. However, most of the publications are based on small numbers of patients (in case reports or case series) and may be affected by bias. Randomised controlled trials provide higher‐quality research findings and allow us to assess the evidence of drug effectiveness with more certainty.
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