3B11.0

ICD-11 MMS 2026-01

Haemophilia B

Description

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%).

Index terms

  • Haemophilia B
  • Christmas disease
  • PTC - [plasma thromboplastin component] deficiency
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  • deficiency of functional factor ix
  • functional factor IX deficiency
  • plasma thromboplastin component deficiency
  • sex-linked factor IX deficiency disease
  • Severe haemophilia B
  • Moderately severe haemophilia B
  • Mild haemophilia B
  • Symptomatic form of haemophilia B in female carriers

Inclusions

  • PTC - [plasma thromboplastin component] deficiency
  • Severe haemophilia B
  • Moderately severe haemophilia B
  • Mild haemophilia B
  • Symptomatic form of haemophilia B in female carriers

Exclusions from above levels

  • JA43.3 – Postpartum coagulation defects
  • 04 – Diseases of the immune system
  • 05 – Endocrine, nutritional or metabolic diseases
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  • 18 – Complications of pregnancy, childbirth or the puerperium
  • 19 – Certain conditions originating in the perinatal period
  • 20 – Congenital malformations, deformations or chromosomal abnormalities
  • 22 – Injury, poisoning or certain other consequences of external causes
  • http://id.who.int/icd/entity/1000704511 – Human immunodeficiency virus disease
  • JB64.1 – Other diseases of the blood or blood-forming organs or certain disorders involving the immune mechanism complicating pregnancy, childbirth or the puerperium

Sibling codes

  • 3B11.Y – Other specified hereditary factor IX deficiency
  • 3B11.Z – Hereditary factor IX deficiency, unspecified

Foundation entity

Haemophilia B – http://id.who.int/icd/entity/1901375668

Allowed postcoordination axes

Part of: 3B11: Hereditary factor IX deficiency › 03: Diseases of the blood or blood-forming organs