Deficiency of plasma plasminogen activator inhibitor 1 results in hyperfibrinolytic bleeding

A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In...

Full description

Saved in:
Bibliographic Details
Published in:Blood 1993-05, Vol.81 (9), p.2357-2362
Main Authors: LEE, M. H, VOSBURGH, E, ANDERSON, K, MCDONAGH, J
Format: Article
Language:English
Subjects:
Biological and medical sciences
Blood Coagulation Factors - analysis
Epistaxis - genetics
Female
Fibrinogen - analysis
Fibrinogen - isolation & purification
Fibrinogen - metabolism
Fibrinolysis - genetics
Hematologic and hematopoietic diseases
Hemorrhage - blood
Hemorrhage - genetics
Humans
Male
Medical sciences
Middle Aged
Pedigree
Plasminogen Activator Inhibitor 1 - blood
Plasminogen Activator Inhibitor 1 - deficiency
Platelet diseases and coagulopathies
Tissue Plasminogen Activator - blood
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A 63-year-old man was evaluated for a lifelong history of bleeding commencing with frequent epistaxis as a child; all previous routine coagulation parameters were within the normal range. The patient's hemorrhagic disorder is characterized predominantly by delayed bleeding at surgical sites. In the resting state, there was no clinical or laboratory evidence of excessive fibrin(ogen)olysis. Bleeding was not caused by disseminated intravascular coagulation, factor XIII deficiency, alpha 2-antiplasmin deficiency, or dysfibrinogenemia. It was found that the patient was deficient in plasma PAI-1 antigen and activity but with approximately half normal antigen and normal activity of platelet PAI-1. The low concentration of plasma PAI-1 was insufficient to neutralize circulating t-PA, resulting in high t-PA activity with normal antigen and causing the hyperfibrinolytic activity observed. Studies on seven family members of the proband indicated autosomal inheritance of plasma PAI-1 deficiency. Studies on this patient emphasize a clear correlation between decreased plasma PAI-1 activity and hyperfibrinolytic bleeding and also emphasize the unique role of plasma PAI-1 in the balance between the coagulation and fibrinolytic mechanisms.
ISSN:0006-4971
1528-0020
DOI:10.1182/blood.V81.9.2357.2357