3B50

ICD-11 MMS 2026-01

Inherited fibrinolytic defects

Description

A disease caused by genetically inherited mutations affecting the fibrinolysis system which prevents blood clots from growing and becoming problematic. This disease is characterised by defects in the fibrinolysis system leading to coagulation of the blood. This disease may present with thrombosis.

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

Child codes

  • 3B50.0 – Congenital alpha-2 antiplasmin deficiency
  • 3B50.1 – Congenital plasminogen activator inhibitor type 1 deficiency
  • 3B50.Y – Other specified inherited fibrinolytic defects
  • 3B50.Z – Inherited fibrinolytic defects, unspecified

Foundation children elsewhere

  • DA0D.3 – Hypoplasminogenaemia

Sibling codes

  • 3B51 – Acquired fibrinolytic defects

Foundation entity

Inherited fibrinolytic defects – http://id.who.int/icd/entity/1502898025

Part of: 03: Diseases of the blood or blood-forming organs