HEMOPHILIA B LEYDEN is a rare form of FIX deficiency
Here are some key points to be noted regarding Hemophilia B Leyden.
- It is a rare form of FIX deficiency, hemophilia B Leyden, undergoes postpubertal phenotypic resolution.
- Patients with this condition present with hemophilia B in early childhood, with FIX activity ranging from less than 1% to 13% of normal.
- Plasma levels rise to as high as 70% of normal after the onset of puberty with resolution of bleeding complications.
- Mutations in hemophilia B Leyden have been identified in the promoter region of the FIX gene, within which a consensus sequence for steroid receptor binding is located.