Benign Hematology Conditions
Bleeding disorders such as hemophilia are a group of conditions marked by abnormal or excessive bleeding and/or bruising. These disorders include some rare diseases as well as some very common conditions. Most bleeding disorders are inherited genetically, but individuals can acquire a bleeding disorder as a result of illness.
Bleeding Disorder SymptomsA bleeding disorder can come to light in several different ways. People with bleeding disorders can experience symptoms like easy bruising or frequent nosebleeds. Excessive menstrual bleeding may indicate an underlying bleeding disorder. Others may experience excessive or unexpected bleeding during surgery or a dental extraction. For some patients, minor injuries can lead to bleeding. Patients with bleeding disorders can develop anemia.
Types of Bleeding DisordersThe body has complex systems for preventing excessive bleeding. The two main parts of this system are blood platelets (which are produced in the bone marrow) and proteins called coagulation factors or clotting factors (mainly produced by the liver). Any defect in the production of platelet cells or clotting factors can lead to bleeding problems. Many bleeding disorders are inherited genetically, but some are acquired because of another medical illness such as liver disease.
HemophiliaPeople with hemophilia have reduced levels of specific clotting factors in their blood. The disorder is usually inherited, and it affects only males. Approximately 20,000 men in the U.S. have hemophilia. There are two forms — type A hemophilia (reduced factor VIII) and type B hemophilia (reduced factor IX). Symptoms range from mild to severe, depending on the level of clotting factor present. Severe hemophilia can lead to joint, muscle and organ damage.
Von Willebrand DiseaseVon Willebrand disease (VWD) affects up to 1 percent of the population, including women and men. Many people who have the condition are undiagnosed. Generally inherited, VWD is marked by problems with Von Willebrand factor, a clotting protein that helps blood platelets stick to the walls of injured blood vessels. VWD is often mild in comparison to hemophilia. Women with VWD may experience heavy menstrual periods. Learn more about pregnancy, menstruation and blood disorders.
Platelet Function DisordersThere are several disorders of the blood platelets that lead to bleeding problems. For example, a patient’s platelets may be less able to stick to the blood vessel wall to plug an injury. Most platelet function disorders are mild, but some can lead to severe bleeding.
Bleeding Disorder TreatmentsThere are three general treatments for bleeding disorders: risk reduction, medications, and replacement therapy.
Risk reductionPatients with bleeding disorders should avoid medications that thin the blood. They may also need to make some changes to lifestyle or activities to reduce their risk of bleeding. For patients who require surgery, physicians can take steps to reduce the risk of operative and post-operative bleeding. Learn more about blood disorder surgical concerns.
MedicationsSeveral drugs are available that improve blood coagulation. Desmopressin acetate, often given as a nasal spray, can increase the body’s production of certain clotting factors. Other drugs help prevent clots from dissolving.
Replacement therapyPatients with moderate to severe bleeding disorders may require transfusion of blood platelets or clotting factors. Clotting factors may be donated human blood products or lab-synthesized proteins. Patients with severe bleeding disorders may receive clotting factor transfusions as a preventive measure.
Date: Sept. 28, 2012
Online Editor(s): Richard Petre