Acquired hemophilia is a rare but potentially life-threatening bleeding disorder caused by the development of autoantibodies directed against plasma coagulation factors, most frequently factor VIII (FVIII). Autoantibodies against other factor proteins have also been reported.
Besides, Why is hemophilia B called Christmas disease?
Hemophilia B is also known as Christmas disease. It is named after the first person to be diagnosed with the disorder in 1952, Stephen Christmas. As the second most common type of hemophilia, it occurs in about 1 in 25,000 male births and affects about 4,000 individuals in the United States.
Keeping this in mind, Is hemophilia congenital or acquired? Commonly, the term hemophilia is used to denote the inherited bleeding disorder that affects mostly males from birth. This is called congenital or inherited hemophilia.
Can hemophilia B be acquired?
Acquired hemophilia B is caused by the body’s production of antibodies against its own factor IX protein. The factor IX antibodies destroy circulating factor IX in the blood causing bleeding symptoms. Acquired hemophilia B is extremely rare; most cases of acquired hemophilia are in those with hemophilia A.
Can acquired hemophilia be passed down?
The mutation causes the body to produce too little factor VIII or IX. This change in a copy of the gene making factor VIII or factor IX is called a hemophilia allele. Most people who have hemophilia are born with it. It almost always is inherited (passed down) from a parent to a child.
What is hemophilia called Christmas disease?
Hemophilia B, also known as factor IX deficiency or Christmas disease, is the second most common type of hemophilia. The disorder was first reported in the medical literature in 1952 in a patient with the name of Stephen Christmas. The most famous family with hemophilia B was that of Queen Victoria of England.
Why is clotting factor IX called Christmas factor?
The clotting factor IX is also known as Christmas factor. The name is derived from the boy, Stephen Christmas. He was lacking this factor and the deficiency led him to acquire hemophilia. The Christmas factor is produced from the liver.
Is hemophilia considered a rare disease?
Acquired hemophilia is a rare autoimmune disorder characterized by bleeding that occurs in patients with a personal and family history negative for a bleeding disorder.
What is acquired VIII deficiency?
Acquired factor VIII deficiency is a bleeding disorder that requires prompt diagnosis and management to avert severe, life-threatening bleeding and death. Despite knowledge of this disorder of coagulation for several decades, relatively little is still known about this disease because of its rare incidence.
What is acquired coagulation deficiency?
The most common causes of acquired coagulation disorders are liver disease, vitamin K deficiency, and disseminated intravascular coagulation (DIC). More rarely, inhibitors, external factors such as drugs or extracorporeal circulation, or other diseases such as amyloidosis are present.
How do you get hemophilia B?
Hemophilia B is caused by an inherited X-linked recessive trait, with the defective gene located on the X chromosome. Females have two copies of the X chromosome. If the factor IX gene on one chromosome does not work, the gene on the other chromosome can do the job of making enough factor IX.
Is Christmas disease hereditary?
A person is born with Christmas disease, but it may not be diagnosed until later in life. It’s estimated that two-thirds of cases are inherited. The other cases are caused by spontaneous gene mutations that occur for unknown reasons during fetal development. The disease almost exclusively in males.
How rare is acquired haemophilia?
Acquired hemophilia is an extremely rare condition affecting around one in a million. It’s usually diagnosed when patients present prolonged bleeding, or extensive bruising on the truck, legs and arms which looks very different from usual injury-related bruising.
Can two normal parents produce a hemophiliac son?
It is also possible for all the children in the family to inherit the normal gene or all to inherit the hemophilia gene. Figure 2-3. For a mother who carries the hemophilia gene, the chances of giving birth to a child with hemophilia are the same for each pregnancy.
Can hemophilia be passed from father to daughter?
A father who has hemophilia possesses the gene and passes it on to his daughter because daughters receive two X chromosomes, one from their mother and one from their father. This is why daughters of men with hemophilia are called obligate carriers.
Which blood clotting factor is known as Christmas factor?
Hemophilia B is the second most common type of hemophilia. It is also known as factor IX deficiency, or Christmas disease.
What are some other names for hemophilia?
Other Names for Hemophilia
- Classic hemophilia.
- Factor VIII deficiency.
What does Hemarthrosis mean?
Hemarthrosis is a condition of articular bleeding, that is into the joint cavity. This can occur after an injury or, more commonly, in bleeding disorders such as hemophilia. Patients will typically present with pain, swelling and a decreased range of motion of the involved joint.
Why do they call it Christmas disease?
Hemophilia B is called Christmas Disease because it was named for a 5 year old boy named Christmas. He was the first patient described in a case series of 7 patients by Biggs et.
What is the Christmas factor in blood clotting?
Christmas disease, also called hemophilia B or factor IX hemophilia, is a rare genetic disorder in which your blood doesn’t clot properly. If you have Christmas disease, your body produces little or no factor IX. This leads to prolonged or spontaneous bleeding.
What is the function of Christmas factor?
The role of Christmas Factor is to cleave a peptide bond in another enzyme, Factor X. In doing so, it activates this enzyme which is the next step of the clotting cascade that ultimately results in cross-linked fibrin and a blood clot.
How common is hemophilia in the world?
The worldwide incidence of hemophilia A is approximately 1 case per 5000 males, with approximately one third of affected individuals not having a family history of the disorder. The prevalence of hemophilia A varies with the reporting country, with a range of 5.4-14.5 cases per 100,000 males.
What is the average life expectancy of a person with hemophilia?
In severe hemophilia, all-cause mortality did not change significantly during 1977 to 1999. During this period, it exceeded mortality in the general population by a factor of 2.69 (95% confidence interval [CI]: 2.37-3.05), and median life expectancy in severe hemophilia was 63 years.
What race is hemophilia most common in?
The average age of persons with hemophilia in the United States is 23.5 years. Compared to the distribution of race and ethnicity in the U.S. population, white race is more common, Hispanic ethnicity is equally common, while black race and Asian ancestry are less common among persons with hemophilia.