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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

Last reviewed: March 3, 2013.

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

  1. Kessler C. Hemorrhagic disorders: Coagulation factor deficiencies. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 180.
  2. 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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The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is an independent audit to verify that A.D.A.M. follows rigorous standards of quality and accountability. A.D.A.M. is among the first to achieve this important distinction for online health information and services. Learn more about A.D.A.M.'s editorial policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only — they do not constitute endorsementscof those other sites. © 1997–2011 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.

Copyright © 2013, A.D.A.M., Inc.

What works?

  • Desmopressin acetate (DDAVP) for preventing and treating acute bleeds during pregnancy in women with congenital bleeding disorders
    Congenital bleeding disorders cause problems with bleeding during pregnancy, labour and delivery. Bleeding complications in women with congenital bleeding disorders are an important cause of disease and death linked to childbirth. Agents to stop the flow of blood are used for women with these bleeding disorders during pregnancy. Desmopressin acetate is a drug used to effectively increase the concentration of factor VIII in the blood and to increase the clumping together of platelets to stop bleeding. It does not come from human plasma and it carries no risk of infection. It might be a precious resource in patients with von Willebrand disease, haemophilia A or congenital platelet disorders to prevent and treat bleeding episodes related to pregnancy. We did not find any randomised controlled trials assessing desmopressin acetate in this group of patients. Given the ethical considerations, future randomised controlled trials are unlikely. Evidence is needed to show the risks and benefits of desmopressin acetate when used to prevent and treat bleeding during pregnancy in women with congenital bleeding disorders. While there is evidence from observational trials that shows the drug is effective in stopping and preventing bleeding, we conclude that there is still a need to generate other high quality controlled evidence.
See all (4) ...

Figures

  • Blood clots.

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