Hemophilia B, or hemophilia B, is a recessive genetic disease linked to the sex chromosome, X, and causes a deficiency of clotting factor IX. Clotting factors work to form fibrin tissue that stops bleeding in the event of an injury. When suffering from hemophilia B, the blood clotting process is disrupted, which means that the injured person will bleed for a long time if he suffers a wound or scratch.
For more: Hemophilia patient infographic
Hemophilia B patients constitute only 20% of all hemophilia patients.
What is hereditary hemorrhagic disease?
Hereditary bleeding disease, or hemophilia, is one of the types of bleeding disorders that causes a defect in the natural blood clotting mechanism and slows down the rate of blood clotting, which may lead to things such as:
Ease of bleeding.
Bleeding continues for a longer period than normal when sustaining any wound.
This disease results from a deficiency in the levels of special types of proteins responsible for clotting processes, called clotting factors.
Bleeding from some simple or moderately severe wounds may make it a health problem that must be dealt with medically, as it is difficult for the body to stop the bleeding through the usual clotting processes.
In some cases, the bleeding occurring in people with hereditary hemorrhagic disease may get out of control, which may cause a group of severe complications that may pose a threat to the patient’s life, especially if the bleeding occurs in the joints or in one of the internal organs.
There are many treatments that may help the patient keep the patient's condition under control to reduce his chances of serious bleeding.
It should be noted that the incidence of hereditary hemophilia is limited to men only in the majority of cases, but if the type of disease is hemophilia type C, this disease may affect women as well.
Types of hereditary hemorrhage disease
The human body contains several types of clotting factors, the deficiency of each of which causes a certain type of hereditary hemorrhagic disease, as follows:
Hemophilia type A: One of the most common types of hereditary bleeding disorders. It is caused by a deficiency of clotting factor VIII. This type of disease is very severe and often causes serious bleeding in the large joints.
Hemophilia type B: This type results from a deficiency of clotting factor IX, and the severity of this type of disease ranges from mild to serious.
Hemophilia type C: This type results from a deficiency of clotting factor XI, and differs in the way it is transmitted genetically from the previous two types. Therefore, unlike the previous types, this type of hemophilia may affect women.
Causes of hereditary hemorrhage
Exactly as the name suggests, the cause of this disease is a genetic defect that is often inherited from the mother’s genes to the child, but while females often carry the disease gene without contracting it, only males are infected with the disease in the majority of cases and show its symptoms.
In cases of acquired hemorrhagic disease, this type arises as a result of a different cause, as the body begins to produce antibodies that attack the clotting factors present in the body, which prevents them from performing their normal functions.
Symptoms of hereditary hemorrhagic disease
Here is a list of the most prominent symptoms expected to appear in patients with hereditary hemorrhage:
Bleeding gums.
Muscle pain that may result from internal bleeding.
Swelling and pain in the joints may result from local internal bleeding in the joint area.
Prolonged nosebleeds for no apparent reason.
Bleeding in the digestive tract.
Bruises covering large areas of skin.
Prolonged bleeding that does not stop quickly after undergoing medical procedures such as: surgeries and injections.
Blood in the urine or blood in the stool.
Brain hemorrhage - in rare cases - symptoms include the following: severe and prolonged headache, frequent vomiting, double vision, convulsions, and general weakness.
Heavy menstrual cycle in women, which is a symptom that may appear in some uncommon cases in which the disease may affect women.
It should be noted that the severity of the apparent symptoms varies depending on the levels of clotting factors present in the body and the extent of their deficiency, and varies depending on the type of hereditary bleeding disease.
Diagnosis and treatment of hemorrhagic disease
The condition is usually easily diagnosed once the baby is born, either by investigating the cause of abnormal bleeding after the baby is born or by knowing the parents' medical history.
There is no known cure for this disease, but it can be kept under control through some treatment options, such as:
Hormonal therapy: This treatment may help stimulate the body to produce larger amounts of clotting factors and raise their percentage in the body.
Replacement therapy for clotting factors: It is the main treatment for severe types of hereditary hemorrhagic disease.
Thrombolytic medications: These medications help slow the rate of disintegration and dissolution of blood clots.
