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Coagulopathy
Bleeding Disorders

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What is Bleeding Disorders

A bleeding disorder exists when it is hard for a person to stop bleeding. When a person bleeds, the blood clots, stopping the bleeding. The clotting process, also called coagulation, changes blood from a liquid to a solid state. It takes both blood cells (called platelets) and proteins (called clotting factors) for blood to clot properly. When a person has a bleeding disorder, the blood platelets are not working like they should, or there are not enough platelets or clotting factors in the blood. This causes a person to bleed for longer periods of time than normal. Bleeding disorders tend to run in families and can be hard to detect in women. These disorders can be controlled, so a person can have a healthy and full life.

Are there different types of bleeding disorders?

Yes, there are different types of bleeding disorders a person can have. The two most common types of bleeding disorders are:

  • Hemophilia - while this well-known disease affects men and women, most people who have hemophilia are men. Even though most people think hemophiliacs bleed to death from cuts, they actually suffer problems with internal bleeding into the joints, muscles, and organs. Treatment involves replacing the missing clotting factor in a person's blood, done through intravenous (IV) treatment, where a small tube is inserted into a vein.

  • von Willebrand Disease (VWD) - a lesser known bleeding disorder that affects both women and men, VWD is the most common bleeding disorder in women. This disease causes bleeding in the intestines, bleeding gums, and very heavy menstrual periods in women. Treatment involves the release of stored clotting factors in the blood, or in severe cases replacing the clotting factor through IV treatment or with a nasal spray. When a person with VWD has a dental procedure or dental surgery, they need to take medication beforehand to reduce bleeding. Over the past 20 years, researchers have made great progress in diagnosing and treating VWD.

Is heavy bleeding during menstruation a bleeding disorder?

Heavy bleeding, or menorrhagia, during menstruation is not necessarily caused by a bleeding disorder. But, heavy bleeding can be a symptom of the bleeding disorder von Willebrand Disease (VWD). When a woman has heavy bleeding, it can be hard to find the exact cause of the bleeding. Certain gynecological diseases, using an intrauterine device for birth control, and taking medications can also cause heavy bleeding in women.

What are the symptoms of bleeding disorders? How would I know if I had one of these disorders?

Symptoms of bleeding disorders include:

  • Very heavy bleeding with menstrual periods (menorrhagia)
  • Unusual bleeding after injury or surgery
  • Bleeding from small cuts that starts and stops over several hours
  • Frequent or prolonged nosebleeds
  • Unusual bleeding from the mouth or gums after a tooth extraction

If you have any of the following symptoms, you should discuss them with your health care provider. Your doctor may order tests to rule out a bleeding disorder, including a test for VWD. Be aware that your test results could be affected by your menstrual cycle. Because of this, tests may need to be done at different points in your menstrual cycle. Also know that just because your mother or your sister may also have had heavy periods, this may not be normal for you. If you are having heavy periods with no known reason, you need to be tested for VWD. Not all health care providers test for VWD when a woman is having heavy bleeding. In December 2001, the American College of Obstetrics and Gynecologists (ACOG) issued a report to health care providers to consider VWD as a possible cause for heavy menstrual bleeding in women. ACOG now recommends testing for VWD when:

  • a woman is having heavy menstrual bleeding with no known cause;
  • an adolescent is having heavy menstrual bleeding without other cause (testing should be done before starting any hormone therapy like birth control pills);
  • hysterectomy for heavy menstrual bleeding is being considered.
Why is it important to know if I have a bleeding disorder?

When a bleeding disorder is not diagnosed and treated, it can cause serious problems, some of which can be life threatening. Women can have severe bleeding during and after childbirth or with a miscarriage. Severe bleeding can also happen during or after dental or other kinds of surgery, and after an injury or accident. A person can suffer internal bleeding, as well as bleeding in the brain. If you know that you have a bleeding disorder, be sure to tell your health care providers, especially before dental procedures, surgery, pregnancy and childbirth.

How are bleeding disorders treated?

The treatment for bleeding disorders varies. Disorders that cause minor bleeding problems may need no treatment. Medications are available to prevent or treat more severe bleeding problems, such as heavy periods or extreme bleeding during dental or surgical procedures. Birth control pills are frequently used to treat heavy bleeding in women with VWD. Nasal sprays, like desmopressin acetate (DDAVP), are used for both heavy menstrual bleeding and nosebleeds. A form of DDAVP can also be injected into a person's vein before surgery or if they are having serious bleeding. Medicines to clot the blood, called clotting agents, help control bleeding. For women who have extreme menstrual bleeding that does not go away with medication, sometimes surgery is an option. Endometrial ablation can be done, which applies heat, laser, or radio waves to the inside of the uterus, or womb, to control bleeding. Hysterectomy, or removal of the uterus may also be done to stop bleeding. But, both of these surgeries, for women with bleeding disorders, increase the risk of bleeding from the surgery itself.

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What is Immune Thrombocytopenic Purpura

Immune Thrombocytopenic Purpura (ITP) is a disorder of the blood.

Immune refers to the immune system's involvement in this disorder. Antibodies, part of the body's immunologic defense against infection, attach to blood platelet, cells that help stop bleeding, and cause their destruction. Thrombocytopenia refers to decrease in blood platelet. Purpura refers to the purplish- looking areas of the skin and mucous membranes (such as the lining of the mouth) where bleeding has occurred as a result of decreased platelet.

Some cases of ITP are caused by drugs, and others are associated with infection, pregnancy, or immune disorders such as systemic lupus erythematosus. About half of all cases are classified as "idiopathic," meaning the cause is unknown.

What Are the Symptoms of ITP?The main symptom is bleeding, which can include bruising ("ecchymosis") and tiny red dots on the skin or mucous membranes ("petechiae"). In some instances bleeding from the nose, gums, digestive or urinary tracts may also occur. Rarely, bleeding within the brain occurs.

How Is ITP Diagnosed?The physician will take a medical history and perform a thorough physical examination. A careful review of medications the patient is taking is important because some drugs can be associated with thrombocytopenia. A complete blood count will be done. A low platelet count will establish thrombocytopenia as the cause of purpura. Often the next procedure is a bone marrow examination to verify that there are adequate platelet-forming cells (megakaryocyte) in the marrow and to rule out other diseases such as metastatic cancer (cancer that has spread to the bone marrow) and leukemia cancer of the blood cells themselves). Another blood sample may be drawn to check for other conditions sometimes associated with thrombocytopenia such as lupus and infection.

Acute and Chronic Form of Thrombocytopenic Purpura

Acute (temporary) thrombocytopenic purpura is most commonly seen in young children. Boys and girls are equally affected. Symptoms often, but do not necessarily, follow a viral infection. About 85 percent of children recover within 1 year and the problem doesn't return.

Thrombocytopenic purpura is considered chronic when it has lasted more than 6 months. The onset of illness may be at any age. Adults more often have the chronic disorder and females are affected two to three times more than males. The onset of illness may be at any age.

How Is ITP Treated?

If the doctor thinks a drug is the cause of the thrombocytopenia, standard treatment involves discontinuing the drug's use. Infection, if present, is treated vigorously since control of the infection may result in a return of the platelet count to normal.

The treatment of idiopathic thrombocytopenic purpura is determined by the severity of the symptoms. In some cases, no therapy is needed. In most cases, drugs that alter the immune system's attack on the platelet are prescribed. These include corticosteroids (i.e., prednisone) and/or intravenous infusions of immune globulin. Another treatment that usually results in an increased number of platelet is removal of the spleen, the organ that destroys antibody-coated platelet. Other drugs such as vincristine, azathioprine (Imuran), Danazol, cyclophosphamide, and cyclosporine are prescribed for patients only in the severe case where other treatments have not shown benefit since these drugs have potentially harmful side effects.

Except in certain situations, (e.g., internal bleeding and preparation for surgery), platelet transfusions usually are not beneficial and, therefore, are seldom performed. Because all therapies can have risks, it is important that overtreatment (treatment based solely on platelet counts and not on symptoms) be avoided. In some instances lifestyle adjustments may be helpful for prevention of bleeding due to injury. These would include use of protective gear such as helmets and avoidance of contact sports in symptomatic patients or when platelet counts are less than 50,000. Otherwise, patients usually can carry on normal activities, but final decisions about activity should be made in consultation with the patient's hematologist.

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Hereditary Bleeding Disorders

Hemophilia

Hemophilia is an inherited bleeding disorder that affects 18,000 persons (primarily males) in the United States. The disorder results from deficiencies in blood clotting factors and can lead to spontaneous internal bleeding and bleeding following injuries or surgery. These bleeding episodes can cause severe joint damage, neurological damage, damage to other organ systems involved in the hemorrhage, and, in rare cases, death. Treating the bleeding episodes involves the prompt and proper use of clotting factor concentrates.

von Willebrand disease

The most common bleeding disorder is von Willebrand disease (vWD), which is found in approximately 1-2% of the U.S. population. VWD results from a deficiency or defect in the body's ability to make von Willebrand factor, a protein that helps blood clot. Although VWD occurs in men and women equally, women are more likely to notice the symptoms because of heavy or abnormal bleeding during their menstrual periods and after childbirth.

Prevention activities

  • CDC helps support a network of hemophilia treatment centers (HTCs). This network promotes the management, treatment, and prevention of complications experienced by persons with hemophilia and other hereditary bleeding disorders.

  • CDC has established a surveillance system, the Universal Data Collection project, to monitor blood safety and to conduct research on health-care outcomes. The system is integrated into the HTC network.

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Bleeding Disorders