Posted in Hematology, Hematopathology

HEMOPHILIA B LEYDEN- A rare form of FIX deficiency

HEMOPHILIA B LEYDEN is a rare form of FIX deficiency

Here are some key points to be noted regarding Hemophilia B Leyden.

  1. It is a rare form of FIX deficiency, hemophilia B Leyden, undergoes postpubertal phenotypic resolution.
  2. Patients with this condition present with hemophilia B in early childhood, with FIX activity ranging from less than 1% to 13% of normal.
  3. Plasma levels rise to as high as 70% of normal after the onset of puberty with resolution of bleeding complications.
  4. 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.

REVIEW QUESTION- A QUESTION FROM DM HEMATOPATHOLOGY PGIMER ENTRANCE

CLICK TO REVEAL THE ANSWER

ANSWER is C: Steroid receptor is involved in the pathogenesis.

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