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Why is hemophilia B less common than hemophilia A, but just as dangerous?
Hemophilia B, also known as Christmas disease, is mainly caused by a genetic defect in coagulation factor IX, which leads to abnormal blood coagulation and prone to congestion and bleeding. Although the incidence of hemophilia B is lower than that of hemophilia A, its hidden risks worry experts and patients alike.
"Hemophilia B is unevenly distributed between men and women and mainly affects men, which is also one of its dangers."
Background of hemophilia
There are many types of hemophilia, the most common of which are hemophilia A and hemophilia B. According to statistics, the incidence of hemophilia B is approximately one in every 40,000 people, accounting for only 15% of all hemophilia patients. It has a history of several decades and was first identified as an independent disease entity in 1952.
Symptoms and complications
The main symptoms of hemophilia B include: prone to congestion, urethral bleeding, nosebleeds, and bleeding in the joints. Due to the fear of bleeding, patients often neglect oral hygiene, leading to an increased incidence of periodontal disease and tooth decay. People with severe hemophilia may even experience spontaneous bleeding that affects various tissues in the mouth.
"Patients with hemophilia B will experience many oral bleeding episodes throughout their lives, requiring treatment for bleeding an average of 29.1 times per year."
Genes and pathophysiology
Hemophilia B is an X-linked recessive genetic disorder that primarily affects males. Due to the lack of coagulation factor IX, patients have poor hemostatic ability after injury and are prone to bleeding. According to research, Factor IX is unable to properly initiate the blood clotting process when someone experiences minor trauma.
Diagnosis and treatment options
Methods for diagnosing hemophilia B include coagulation screening, bleeding scores, and coagulation factor testing. Treatment relies primarily on intravenous administration of factor IX, with supplementation in cases of major bleeding. With the advancement of science and technology, the gene therapy Etranacogene dezaparvovec (Hemgenix) was approved by the US FDA in 2022, bringing new hope to patients with hemophilia B.
"During surgery, necessary measures should be taken to reduce the risk of bleeding, such as using hemostatic drugs."
Latest developments in research
In the field of gene therapy, FLT180 is a new treatment candidate in development that shows potential to restore clotting factor IX. However, the success of this therapy needs to avoid interference from the immune response, and further research is needed.
Medical and socio-cultural impact
Historically, hemophilia B has had unique cultural significance because many members of European royal families were affected by the disease, leading to hemophilia B being called the "royal disease." In many countries, the history of the disease and the experiences of patients have caused society to pay more and more attention to blood safety and treatment convenience.
Even though there are fewer cases of hemophilia B than hemophilia A, its impact on patients' lives is obvious. However, with the advancement of modern medicine, patients' lives are getting better and better protected, and the treatment options they face are also increasing. How will the future evolve, and how should we deal with the challenges of hemophilia B?
