ICD-11 classes
03 Diseases of the blood or blood-forming organs
Coagulation defects, purpura or other haemorrhagic or...
Coagulation defects
Congenital or constitutional haemorrhagic condition
3B11 — Hereditary factor IX deficiency

ICD-11 3B11 — Hereditary factor IX deficiency

A disease caused by a genetically inherited X-linked recessive trait leading to a defective gene located on the X chromosome. This disease is characterised by low levels of the protein factor IX in the body leading to increased haemorrhaging and bruising due to clotting abnormalities. Confirmation is by identification of recessive trait by genetic testing.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 3B11 contains 3 clarifying diagnoses:

  1. 3B11.0 — Haemophilia B
  2. 3B11.Y — Other specified hereditary factor IX deficiency
  3. 3B11.Z — Hereditary factor IX deficiency, unspecified

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