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Hemophilia B

Definition

What is hemophilia type B?

Hemophilia B is a blood clotting disorder that results in bleeding that lasts longer than normal. This disease is caused by a lack of blood clotting factor or coagulation IX (nine) in the body.

Coagulation factors are proteins that play a role in the process of blood clotting when there is injury or bleeding. The human body has about 13 types of blood clotting factors which work with platelets to clot the blood. When one of the clotting factors is reduced, the blood clotting process cannot occur completely.

Hemophilia is a disease that consists of several types, namely A, B, and C. What distinguishes each type of hemophilia is which type of clotting factor is reduced from the body.

This disease, which is also called Christmas disease, is a genetic disease, aka hereditary. However, there are also cases where hemophilia is acquired (acquired), not lowered.

How common is this disease?

Reporting from the site Indiana Hemophilia and Thrombosis Center, hemophilia B is found in 1 in 25,000 newborns. The incidence of this type of hemophilia is 4 times less than that of hemophilia A.

Although this disease is hereditary, about 1/3 of cases occur without heredity.

Signs and symptoms

What are the signs and symptoms of hemophilia type B?

The most common signs and symptoms of hemophilia B are not very different from other types of hemophilia. The common symptoms of hemophilia type B are nosebleeds and frequent bruising on several parts of the body. In more severe or severe cases, sufferers may experience bleeding spontaneously or without cause.

Spontaneous bleeding generally occurs in tissues in the body, such as joints and muscles. This condition causes pain, swelling, and difficulty moving. Bleeding in the joint is called hemarthrosis.

When should I go to the doctor?

Seek medical attention immediately if you or someone else has:

  • symptoms of bleeding in the brain (severe headache, vomiting, decreased consciousness)
  • accidents that make it difficult for blood to stop flowing
  • joints that are swollen and feel warm to the touch

If your family or parents have a history of hemophilia, you will also need to undergo genetic testing to see if there is a risk of this disease in your body.

Causes and risk factors

What are the causes and risk factors for hemophilia B?

The reduction in blood clotting factor VIII in hemophilia B is caused by a genetic mutation that is usually inherited from the parent.

However, there is also a possibility that this disease may arise even if the patient does not have a parent who has hemophilia. According to the site Genetic Home Reference, a condition called acquired hemophilia this can occur due to pregnancy, immune system disorders, cancer, allergic reactions to drugs, or other unknown causes.

So, it can be concluded that the risk factor that plays the biggest role in hemophilia B is having a parent with a history of hemophilia or carrying a mutated gene in the body.

Therefore, the only prevention for developing hemophilia is to undergo prenatal test so that the chances of having offspring with this disease can be minimized.

Diagnosis and treatment

The information provided is not a substitute for medical advice. ALWAYS consult your doctor.

What tests are done to diagnose this condition?

Generally, hemophilia B and other types of hemophilia will be immediately investigated in newborns, especially if the parents have a history of hemophilia.

This disease can be diagnosed through a clotting factor concentration test, which aims to determine the amount of blood clotting factors in the body.

It is not uncommon for someone to find out that he has hemophilia after undergoing a surgical procedure or an accident. This could be because he has mild hemophilia, or does not feel symptoms of hemophilia until he is an adult.

What are the treatment options for hemophilia B?

Like other types of hemophilia, hemophilia B also cannot be cured. The existing hemophilia treatment only aims to control the symptoms and reduce the severity of the bleeding that occurs.

Treatment for hemophilia B is similar to hemophilia type A, in that it aims to prevent severe bleeding by giving an injection containing a blood clotting factor concentrate. Even so, the trademarks used for medicine are usually different.

This injection can be done alone at home. In severe cases, sufferers may have to undergo regular injection treatment.

However, treatment can increase a patient’s risk of developing complications from hemophilia, namely inhibitors. Inhibitors are conditions in which the body’s immune system turns against blood clotting factors in the body.

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