ICD-11 classes
03 Diseases of the blood or blood-forming organs
Coagulation defects, purpura or other haemorrhagic or...
Fibrinolytic defects
3B50 — Inherited fibrinolytic defects
3B50.1 — Congenital plasminogen activator inhibitor type 1 deficiency

ICD-11 3B50.1 — Congenital plasminogen activator inhibitor type 1 deficiency

Congenital plasminogen activator inhibitor type 1(PAI-1) deficiency is a disorder that causes premature lysis of haemostatic clots and a moderate bleeding syndrome. Spontaneous bleeding is rarely observed, whereas moderate haemorrhages of the knees, elbows, nose and gingiva are usually triggered by mild trauma. However, menstrual bleeding may be severe and a prolonged bleeding after surgery is common. The PAI-1 deficiency may be qualitative or quantitative, total or partial.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.