3B10.1

ICD-11 MMS 2026-01

Hereditary factor VIII deficiency with anti-factor VIII inhibitor

Description

A disease caused by a genetically inherited mutation leading to a deficiency in clotting due to lack of factor VIII. This disease also causes anti-factor VIII inhibitor antibodies to be produced when receiving transfusions. Anti-factor VIII inhibitor antibodies develop as the body recognises the factor VIII as foreign, therefore deeming factor VIII infusions ineffective. This disease is characterised by increasing haemorrhaging and bruising. Confirmation is by identification of mutations by genetic testing.

Index terms

  • Hereditary factor VIII deficiency with anti-factor VIII inhibitor

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

  • 3B10.0 – Haemophilia A
  • 3B10.Y – Other specified hereditary factor VIII deficiency
  • 3B10.Z – Hereditary factor VIII deficiency, unspecified

Foundation entity

Hereditary factor VIII deficiency with anti-factor VIII inhibitor – http://id.who.int/icd/entity/765707742

Part of: 3B10: Hereditary factor VIII deficiency › 03: Diseases of the blood or blood-forming organs