Hemophilia A is a hereditary bleeding disorder caused by a lack of blood clotting factor VIII. Without enough factor VIII, the blood cannot clot properly to control bleeding.
Factor VIII deficiency; Classic hemophilia; Bleeding disorder - hemophilia A
When you bleed, a series of reactions take place in the body that helps blood clots form. This process is called the coagulation cascade. It involves special proteins called coagulation, or clotting, factors. You may have a higher chance of excess bleeding if one or more of these factors are missing or are not functioning like they should.
Factor VIII (eight) is one such coagulation factor. Hemophilia A is the result of the body not making enough factor VIII.
Hemophilia A is caused by an inherited X-linked recessive trait, with the defective gene located on the X chromosome. Females have two copies of the X chromosome. So if the factor VIII gene on one chromosome does not work, the gene on the other chromosome can do the job of making enough factor VIII.
Males have only one X chromosome. If the factor VIII gene is missing on a boy's X chromosome, he will have hemophilia A. For this reason, most people with hemophilia A are male.
If a woman has a defective factor VIII gene, she is considered a carrier. This means the defective gene can be passed down to her children. Boys born to such women have a 50% chance of having hemophilia A. Their daughters have a 50% chance of being a carrier. All female children of men with hemophilia carry the defective gene.
Risk factors for hemophilia A include:
Severity of symptoms vary. Prolonged bleeding is the main symptom. It is often first seen when an infant is circumcised. Other bleeding problems usually show up when the infant starts crawling and walking.
Mild cases may go unnoticed until later in life. Symptoms may first occur after surgery or injury. Internal bleeding may occur anywhere.
Symptoms can include:
Treatment includes replacing the missing clotting factor. You will receive factor VIII concentrates. How much you get depends on:
Mild hemophilia may be treated with desmopressin (DDAVP). This medicine helps the body release factor VIII that is stored within the lining of blood vessels.
To prevent a bleeding crisis, people with hemophilia and their families can be taught to give factor VIII concentrates at home at the first signs of bleeding. People with severe forms of the disease may need regular preventive treatment.
DDAVP or factor VIII concentrate may also be needed before having dental extractions or surgery.
You should get the hepatitis B vaccine. People with hemophilia are more likely to get hepatitis B because they may receive blood products.
Some people with hemophilia A develop antibodies to factor VIII. These antibodies are called inhibitors. The inhibitors attack factor VIII so that it no longer works. In such cases, a man-made clotting factor called VIIa can be given.
Craig Kessler is a Hematologist Oncology specialist and a Hematologist in Washington, Washington, D.c.. Dr. Kessler has been practicing medicine for over 50 years and is rated as an Elite doctor by MediFind in the treatment of Hemophilia A. He is also highly rated in 29 other conditions, according to our data. His top areas of expertise are Blood Clots, Hemophilia A, Acquired Hemophilia, Essential Thrombocythemia, and Bone Marrow Aspiration. Dr. Kessler is currently accepting new patients.
Gary Kupfer is a Pediatric Hematologist Oncology specialist and a Pediatrics doctor in Washington, Washington, D.c.. Dr. Kupfer has been practicing medicine for over 34 years and is rated as an Advanced doctor by MediFind in the treatment of Hemophilia A. He is also highly rated in 7 other conditions, according to our data. His top areas of expertise are Fanconi Anemia, Congenital Aplastic Anemia, Aplastic Anemia, and Anemia. Dr. Kupfer is currently accepting new patients.
Michael Guerrera is a Pediatric Hematologist Oncology specialist and a Pediatrics doctor in Rockville, Maryland. Dr. Guerrera has been practicing medicine for over 28 years and is rated as an Advanced doctor by MediFind in the treatment of Hemophilia A. He is also highly rated in 3 other conditions, according to our data. His top areas of expertise are Blood Clots, Hemophilia A, Hemophilia B, and Immune Thrombocytopenia. Dr. Guerrera is currently accepting new patients.
You can ease the stress of illness by joining a hemophilia support group. Sharing with others who have common experiences and problems can help you not feel alone.
With treatment, most people with hemophilia A are able to lead a fairly normal life.
If you have hemophilia A, you should have regular checkups with a hematologist.
Complications may include:
Contact your provider if:
Genetic counseling may be recommended. Testing can identify women and girls who carry the hemophilia gene. Identify women and girls who carry the hemophilia gene.
Testing can be done during pregnancy on a baby in the mother's womb.
Summary: This study is looking at how Mim8 works in people with haemophilia A, who either have inhibitors or do not have inhibitors. Mim8 is a new medicine that will be used to avoid bleeding episodes. Mim8 works by replacing the function of the missing clotting factor VIII (FVIII). When and how often the participants will receive Mim8 in this study depends on the treatment participant receives in the curr...
Summary: This study will test how well a new medicine called concizumab works for participants who have haemophilia A or B with or without inhibitors. The purpose is to show that concizumab can prevent bleeds and is safe to use. Participants will have to inject the study medicine every day under the skin with a pen-injector. The study will last for about 2 years.
Published Date: January 25, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Carcao M, Moorehead P, Lillicrap D. Hemophilia A and B. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 135.
Scott JP, Flood VH. Hereditary clotting factor deficiencies (bleeding disorders). In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Textbook of Pediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 503.