What are bleeding disorders?
Bleeding disorders are a group of abnormalities that lead to problems forming blood clots and prolonged bleeding that is hard to stop. Normally, blood flows through your body in vessels called arteries and veins. When a blood vessel is cut or injured, blood flows out, causing bruising and bleeding. Your body stops the bleeding through a process called hemostasis. Hemostasis normally takes only a few minutes. Ineffective hemostatis leads to spontaneous bleeding and bleeding that will not stop.
Hemostasis includes three major steps that all need to function correctly in order to stop bleeding effectively. First, blood vessels need to constrict or narrow when they are damaged or cut. This is called vasoconstriction. Second, cells called platelets need to form a platelet plug at the site of the vessel injury. Third, a complex system of clotting factors is required to finish the clotting process. If any of these three components fail, or work only partially, your body is not able to make a proper blood clot. This results in prolonged bleeding.
Bleeding disorders can be caused by a wide variety of diseases, disorders and conditions. Some of these causes are hereditary (passed from parent to child through genes) and some are acquired. Inherited or hereditary causes of bleeding disorders include hemophilia and von Willebrand’s disease. Other causes of bleeding disorders may include autoimmune disorders, blood cancers, bone marrow problems, infections, kidney failure, liver disease, and medications.
Because of the wide variety of causes, the exact incidence of bleeding disorders in the United States is not known. Von Willebrand’s disease is the most common bleeding disorder in the United States, affecting approximately 1% to 2% of the population, according to the Hemophilia Federation of America (Source: HFA). Treatment of bleeding disorders depends on the specific disorder.
Bleeding disorders may cause uncontrolled internal or external bleeding. This can lead to serious or life-threatening complications, such as anemia, dangerous blood loss, and shock and damage to vital internal organs, such as the brain. Seek prompt medical care if you, or your child, have symptoms of a bleeding disorder, such as easy or excessive bruising after a minor injury, bleeding gums, nosebleeds, or heavy menstrual bleeding.
Seek immediate medical care (call 911) if you, or someone you are with, have serious symptoms, such as bleeding from a wound that does not stop after a few minutes, blood in the urine or stool, vomiting blood, major rectal bleeding, continued dizziness, or shortness of breath.
What are the symptoms of bleeding disorders?
The primary symptom of bleeding disorders is prolonged or excessive bleeding. This can take many different forms in the body and depends on the specific bleeding disorder. Some signs of bleeding disorders can be found only during a medical examination or with special lab tests.
Bleeding can range in severity from a simple bruise to blood in the urine, stool, or sputum (mucus and phlegm). Bleeding can occur from any body part, including the digestive tract, blood vessels, eyes, brain, and joints.
Common bleeding disorder symptoms include:
Anemia (low red blood cell count)
Bleeding into joints causing arthritis-like symptoms
Blood in semen
Dark, tarry bowel movements
Excessive or easy bruising
Heavy or prolonged bleeding from normal cuts and scrapes or minor dental procedures
Heavy or prolonged menstrual periods
Joint pain or stiffness
Prolonged postpartum bleeding (after childbirth)
Unusually pale skin and mucus membranes, such as in the mouth
Symptoms that might indicate a serious or life-threatening condition
In some cases, bleeding might indicate a serious or life-threatening condition that should be immediately evaluated in an emergency setting. Seek immediate medical care (call 911) if you, or someone you are with, have any of these life-threatening symptoms:
Bleeding from an injury or wound that does not stop within a few minutes
Bloody stools, major rectal bleeding, or dark, black or tarry stool
Bloody or pink-tinged urine
Change in mental status, such as confusion and disorientation
Difficulty breathing or shortness of breath
Dizziness that does not go away
Rigid, board-like abdomen
Severe abdominal pain
Vomiting blood or black material (resembling coffee grounds)
What causes bleeding disorders?
Bleeding disorders are caused by problems with any of the three processes needed for proper hemostasis, or blood clot formation. These processes are vasoconstriction (narrowing of blood vessels), platelet coagulation (platelets gathering together to form a plug), and clotting factor activity.
There are a wide variety of diseases, disorders and conditions that can cause bleeding disorders. Some of them are inherited (passed from parent to child through genes) and some are acquired.
Vascular (blood vessel) causes of bleeding disorders
Vascular problems that may cause bleeding disorders include:
Allergic purpura (thought to be an autoimmune disorder that makes blood vessels leaky)
Ehlers-Danlos syndrome (inherited disorder that makes blood vessels fragile and prone to injury)
Hemorrhagic telangiectasia (inherited disorder causing abnormalities in blood vessels and episodes of severe bleeding)
Platelet-related causes of bleeding disorders
Bleeding disorders can be caused by not having enough platelet cells or by having platelet cells that do not work properly. Platelet cells are vital to the clotting process. A low number of working platelets is called thrombocytopenia. Thrombocytopenia results in a platelet plug that is not complete.
Causes of thrombocytopenia include:
Bone marrow diseases
HIV/AIDS (human immunodeficiency virus/acquired immunodeficiency syndrome)
Immune thrombocytopenic purpura (ITP; an autoimmune disorder that is also known as idiopathic thrombocytopenic purpura)
Inherited or genetic conditions
Medications, including aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), penicillin antibiotics, quinine, sulfa antibiotics, and gold salts
Splenic sequestration (pooling of platelets within the spleen)
Systemic lupus erythematosus (disorder in which the body attacks its own healthy cells and tissues)
Clotting factor-related causes of bleeding disorders
There are as many as 20 different proteins that function in your blood as clotting factors. Clotting factors undergo a complex process that results in the production of a substance called fibrin. Fibrin solidifies, or finishes the clot that was started by the platelet cells forming a plug. The clotting factors depend on each other to function properly. Bleeding disorders can be caused by a clotting factor deficiency.
Causes of clotting factor deficiency include:
Certain bacterial infections
Certain cancer treatments
Disseminated intravascular coagulation (DIC), which also reduces platelets in the blood
Fat malabsorption disorders
Hemophilia A (inherited deficiency of factor VIII)
Hemophilia B (inherited deficiency of factor IX that is also known as Christmas disease)
Medications, including warfarin (Coumadin), heparin, and certain cancer chemotherapies
Other inherited clotting factor deficiencies (factors II, V, VII, X, XII)
Vitamin K deficiency
von Willebrand’s disease (inherited disorder)
A number of factors are thought to increase your chances of developing a bleeding disorder. However, the risks for developing a bleeding disorder are specific to each disorder. Common risk factors include:
Family history of bleeding or bleeding disorder
Male gender (hemophilia A and B)
Presence of other diseases, disorders or conditions known to cause bleeding disorders
Use of anticoagulant medications including warfarin (Coumadin)
How are bleeding disorders treated?
Treatment for bleeding disorders begins with seeking medical care from your healthcare provider. To determine whether you have a bleeding disorder, your healthcare provider will likely draw blood samples for laboratory testing.
Specific treatments for bleeding disorders depend on the specific disorder. Treating the underlying disease, disorder or condition may improve bleeding. Treatment may include:
Antifibrinolytic medications, including aminocaproic acid (Amicar) and tranexamic acid (Cyklokapron), to help prevent the normal breakdown of blood clots after they have formed
Clotting factor replacement to replace a specific clotting factor
Corticosteroids, such as prednisone, to suppress the immune system in people who have developed antibodies that inhibit specific clotting factors (acquired hemophilia)
Desmopressin acetate (DDAVP), which is used to temporarily increase factor VIII clotting activity
Immune therapies to suppress the immune system in people with acquired hemophilia
Plasma transfusions to supplement all the clotting factors. Transfusion of fresh frozen plasma may be used after a bleeding episode or before certain procedures to control bleeding.
Platelet transfusions to increase the number of platelets in the bloodstream
Vitamin supplementation for bleeding disorders caused by vitamin K deficiency
What you can do to improve your bleeding disorder
In addition to following your healthcare provider’s instructions and taking all medications as prescribed, you can lessen the effects of bleeding disorders by:
Eat a balanced, nutritious diet
Having regular checkups with your healthcare provider. These checkups should include testing for blood-borne diseases.
Informing your dentist that you have a bleeding disorder so that he or she can take appropriate precautions to limit bleeding during procedures
Protecting your joints through regular exercise and maintaining a healthy weight
Receiving immunizations for hepatitis A and B
Seeking prompt treatment when bleeding occurs
Complications of untreated or poorly controlled bleeding disorders can be serious and even life threatening. You can best treat your bleeding disorder and lower your risk of complications, or delay the development of complications, by following the treatment plan that you and your healthcare professional design specifically for you.
Over time, bleeding disorders can lead to serious complications including: