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
3B11.0 — Haemophilia B
ICD-11 3B11.0 — Haemophilia B
Haemophilia B is a form of haemophilia characterised by spontaneous or prolonged haemorrhages due to factor IX deficiency. Depending on the extent of the factor IX deficiency, it can be severe (biological activity of factor IX below 1%), moderately severe (activity of factor IX between 1% and 5%), or mild (activity of factor IX between 5 and 40%).
It includes 5 items.
- PTC - [plasma thromboplastin component] deficiency
- Severe haemophilia B
- Moderately severe haemophilia B
- Mild haemophilia B
- Symptomatic form of haemophilia B in female carriers
The diagnosis excludes nothing.
It has no clarifying diagnoses.