What are the rare bleeding disorders?

What are the rare bleeding disorders?

Rare bleeding disorders (RBDs) are inherited deficiencies of coagulation factors as fibrinogen, Factor (F) FII, FV, FVII, combined FV/FVIII, FX, FXI, and FXIII. These disorders have usually a low prevalence in the general population and constitute approximately 3 to 5% of all coagulation disorders.

Is Hemophilia A bleeding disorder?

Hemophilia is usually an inherited bleeding disorder in which the blood does not clot properly. This can lead to spontaneous bleeding as well as bleeding following injuries or surgery.

Is haemophilia common?

The two major forms of hemophilia occur much more commonly in males than in females. Hemophilia A is the most common type of the condition; 1 in 4,000 to 1 in 5,000 males worldwide are born with this disorder. Hemophilia B occurs in approximately 1 in 20,000 newborn males worldwide.

What is the rarest form of hemophilia?

Hemophilia B is a rare genetic bleeding disorder in which affected individuals have insufficient levels of a blood protein called factor IX. Factor IX is a clotting factor.

What is factor VII deficiency?

Factor VII deficiency is a rare bleeding disorder that varies in severity among affected individuals. The signs and symptoms of this condition can begin at any age, although the most severe cases are apparent in infancy. However, up to one-third of people with factor VII deficiency never have any bleeding problems.

How do you approach a bleeding disorder?

The approach to a patient with a bleeding disorder needs a comprehensive detailed history and thorough physical examination. There must be a logical systematic approach and a discriminate use of laboratory investigations to reach the diagnosis and assess severity.

What’s a bleeding disorder?

Bleeding disorders are a group of conditions in which there is a problem with the body’s blood clotting process. These disorders can lead to heavy and prolonged bleeding after an injury. Bleeding can also begin on its own. Specific bleeding disorders include: Acquired platelet function defects.

How is a bleeding disorder diagnosed?

These tests may include: a complete blood count (CBC), which measures the amount of red and white blood cells in your body. a platelet aggregation test, which checks how well your platelets clump together. a bleeding time test, which determines how quickly your blood clots to prevent bleeding.

Why is haemophilia rare in females?

Haemophilia is rare in women due to its inheritance pattern. It is an X-linked recessive trait. Females have two copies of the X chromosome and the heterozygous females for the gene are carriers of the disease. For a female to be haemophilic, she should have two copies of the defective gene.

What is haemophilia A?

Haemophilia A (also known as Classic Haemophilia or Factor VIII deficiency) is the most well-known type of clotting disorder. A specific protein is missing from the blood so that injured blood vessels cannot heal in the usual way.

Why is hemophilia A more common than B?

Rarely, hemophilia C (a deficiency of Factor XI) is encountered, but its effect on clotting is far less pronounced than A or B. Hemophilia A and B are inherited in an X-linked recessive genetic pattern and are therefore much more common in males.

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.

Why is hemophilia a dangerous disease?

Haemophilia is a mostly inherited genetic disorder that impairs the body’s ability to make blood clots, a process needed to stop bleeding. This results in people bleeding longer after an injury, easy bruising, and an increased risk of bleeding inside joints or the brain.

What are the 3 types of hemophilia?

The three forms of hemophilia are hemophilia A, B, and C. Hemophilia A is the most common type of hemophilia, and it’s caused by a deficiency in factor VIII. According to the National Heart, Lung, and Blood Institute (NHLBI) Hemophilia B, which is also called Christmas disease, is caused by a deficiency of factor IX.

Why is hemophilia considered a genetic disorder?

Hemophilia is inherited in an X-linked recessive pattern. A condition is considered X-linked when gene mutation that causes it is located on the X chromosome, one of the two sex chromosomes. In males (who have only one X chromosome), one altered copy of the gene in each cell is enough to cause the condition.