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In hemophilia, blood does not clot properly. This usually happens because your body does not have enough of a certain kind of clotting factor. This makes it harder for bleeding to stop. People with hemophilia may bleed a lot after cuts, during surgery, or even after a fall. Some people have abnormal bleeding inside their bodies for no clear reason.
There are two main types of hemophilia:
Hemophilia usually runs in families and almost always affects males. In rare cases, a person may get a type that does not run in the family. This is called acquired hemophilia, and it affects both males and females.
Hemophilia A and B are caused by a flaw in a part of a gene. This flaw affects how much clotting factor a person has and how well it works.
With acquired hemophilia, clotting factors don't work right because the body makes antibodies that attack them.
Symptoms of hemophilia include:
Some people with milder types of the disease may not have symptoms until later in life. But most of the time, hemophilia symptoms are noticed during infancy or childhood. Symptoms noticed in infants include:
If your doctor thinks that you or your child may have a problem with blood clotting, he or she will take a blood sample. The sample will be used in tests that check for the amount of clotting factor. If the level is low, then more tests will find out the type of hemophilia and how severe it is.
How severe the disease is depends on how much clotting factor is produced and when bleeding most often occurs.
If hemophilia runs in your family and you are planning to have children, ask your doctor about tests that can show if you are a carrier. (Only females can be carriers.) This will allow you to make informed decisions about pregnancy and prenatal care.
Hemophilia can be treated by replacing missing blood clotting factors. This is called clotting factor replacement therapy. Clotting factor concentrate is injected into a vein. Replacement therapy can prevent or treat bleeding episodes.
You may need to take medicines, such as desmopressin (for example, DDAVP or Stimate), that help prevent bleeding. You might take medicines at certain times, such as before you have surgery or dental work. Some people also need pain medicine to help with pain from joint damage.
You can live a normal life with treatment. Hemophilia treatment centers are available at most large medical centers. They are an excellent resource to help you and your family get the best care for this condition.
You can take steps at home to prevent bleeding episodes and improve your health.
Chitlur M, Kulkarni R (2015). Hemophilia and related conditions. In ET Bope, RD Kellerman, eds., Conn's Current Therapy 2015, pp. 846–853. Philadelphia: Saunders.
Other Works Consulted
Chitlur M, Kulkarni R (2013). Hemophilia and related conditions. In ET Bope, RD Kellerman, eds., Conn's Current Therapy 2013, pp. 803–810. Philadelphia: Saunders.
Friedman KD, Rodgers GM (2009). Inherited coagulation disorders. In JP Greer et al., eds., Wintrobe's Clinical Hematology, 12th ed., vol. 2, pp. 1379–1424. Philadelphia: Lippincott Williams and Wilkins.
Hillman RS, et al. (2011). Hemophilia and other intrinsic pathway defects. In RS Hillman et al., eds., Hematology in Clinical Practice, 5th ed., pp. 398–410. New York: McGraw-Hill.
Roberts HR, et al. (2010). Hemophilia A and hemophilia B. In K Kaushansky et al., eds., Williams Hematology, 8th ed., pp. 2009–2029. New York: McGraw-Hill.
Srivastava A, et al. (2013). Guidelines for the management of hemophilia. Haemophilia, 19(1): e1–e47. Also available online: http://www.wfh.org/en/page.aspx?pid=1270.
Current as of: November 7, 2019
Author: Healthwise StaffMedical Review: E. Gregory Thompson, MD - Internal MedicineAdam Husney, MD - Family MedicineMartin J. Gabica, MD - Family MedicineBrian Leber, MDCM, FRCPC - Hematology
Current as of: November 7, 2019
E. Gregory Thompson, MD - Internal Medicine & Adam Husney, MD - Family Medicine & Martin J. Gabica, MD - Family Medicine & Brian Leber, MDCM, FRCPC - Hematology
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