WHAT IT IS?
Bleeding disorders are a group of conditions in which the body has a problem with the blood clotting process. In normal state, if bleeding occurs such as in case of injuries, the clotting mechanism gets initiated and arrests the bleeding. If this mechanism fails it will result in bleeding disorders. It can cause abnormal bleeding both outside and inside the body. Some disorders can drastically reduce the amount of blood in your body.
WHO CAN GET IT
The main principle of clotting mechanism is based on the presence of certain protein in the name of clotting factors and the blood cell mainly the platelets. The person who is having defect in these factor and platelet and other associated illness can be affected by this disorder [Fig: 1].
Figure 1: Deficiency of clotting factor
ARE BLEEDING DISORDERS SERIOUS TO CONSIDER?
Bleeding disorders can be particularly dangerous for women, especially if they’re not treated quickly. Untreated cases can increase the risk of excessive bleeding during childbirth, a miscarriage, or an abortion. Women with bleeding disorders may also experience very heavy menstrual bleeding and associated symptoms.
PREVALANCE IN INDIA:
The incidence of hereditary bleeding disorders may vary according to the country and ethnic origin. Von Willebrand’s disease has emerged as the most common hereditary hemorrhagic disease in the industrialized world. In India the most common is Hemophilia A. Approximately 14,000 people with hemophilia are registered at the Hemophilia Federation of India; however, hemophilia remains under-diagnosed and many cases are not registered because most of them are not aware what it is and how serious it is.
LIST OF COMMON BLEEDING DISORDERS:
There are numerous different bleeding disorders, but the following are the most common ones:
- Hemophilia A and B are conditions that occur when there are low levels of clotting factors in your blood. It causes heavy or unusual bleeding into the joints. Though hemophilia is rare, it can have life-threatening complications.
- Certain factors including factor II, V, VII, X, or XII deficiencies are also related to blood clotting problems or abnormal bleeding problems.
- Von Willebrand’s disease is the most common inherited bleeding disorder. It develops when the blood lacks von Willebrand factor, which helps the blood to clot.
Proteins called clotting factors work with platelets to stop bleeding at the site of an injury. People with hemophilia produce lower amounts of either Factor VIII or Factor IX (Christmas factor) than those without the condition. This means the person will more likely to bleed for a longer time after an injury, and they are more susceptible to internal bleeding as well. Males are affected while females are carriers.
Types: There are two major types of hemophilia,
– Type A
– Type B.
In hemophilia A, there is a lack of clotting factor VIII. About 70 percent of people with hemophilia A have the severe form.
In hemophilia B, also known as “Christmas disease,” the person lacks clotting factor IX. Hemophilia occurs in around 1 in every 20,000 males born worldwide.
Figure 2: bleeding into the joints Figure 3: Blood in urine
Figure 4: Profuse bleeding Figure 5: bruising
VON WILLEBRAND’S DISEASE:
It is another genetic bleeding disorder in which patients are prone to frequent bleeding such as nosebleeds (Fig: 6), bleeding gums (Fig: 7), and excessive menstrual periods. Unlike hemophilia, vWD affects men and women equally. Like hemophilia, the severity of vWD depends upon the level of the blood protein. The lower the level of protein in the blood implies more severe is the bleeding.
Figure 6: bleeding from nose Figure 7: Gum bleeding
HOW CAN BE TESTED?
Diagnosis is done through evaluating both the results of screening test and specific test for the factor deficiency.
FINALLY HOW IT CAN BE TREATED?
- Hemophilia is treated with replacement therapy i.e. replacing the clotting factors
- Clotting factor treatments for replacement therapy can be derived from human blood, or they can be synthetically produced in a laboratory.
- Recombinant clotting factors are now considered the treatment of choice because they reduce the risk of transmitting infections.
- Some patients will undergo regular replacement therapy in order to prevent bleeding. This is called prophylactic therapy.
About the Author
This article has been contributed by the Department of Oral Pathology & Microbiology, Yenepoya Dental College under the Yenepoya (Deemed to be University) established in the year1992. The department with its robust alumni of 3000 under- graduates and 67 Post -graduates students and research scholars has many accolades and achievements to its credit. It strives to provide state of art Oral diagnostics and Molecular Pathology while excelling in research activities and instilling a holistic approach in dental education among students. The department contributes its expertise in fostering interdisciplinary collaboration and providing exemplary education and scientific research.