Moderate hemophilia B
ORPHA:169796· ICD-10 D67
Definition
A moderately severe form of hemophilia B characterized by factor IX deficiency (biological activity 1-5 IU/dL) leading to abnormal bleeding as a result of minor injuries or following trauma, surgery or tooth extraction. Spontaneous hemorrhages are rare. The condition primarily affects males but may also be observed in female carriers of disease-causing mutations.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- X-linked recessive
- Age of onset
- Infancy, Neonatal