Moderately Severe Hemophilia B

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Retrieved
2021-01-23
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Moderately severe hemophilia B is a form of hemophilia B (see this term) characterized by factor IX deficiency leading to abnormal bleeding as a result of minor injuries, or following surgery or tooth extraction.

Epidemiology

Moderately severe hemophilia B accounts for around 30% of all cases of hemophilia B.

Clinical description

The biological activity of factor IX is between 1% and 5%. Spontaneous hemorrhages are rare.

Etiology

The disorder is caused by mutations in the F9 gene (Xq28) encoding coagulation factor IX.

Genetic counseling

Transmission is X-linked recessive.