|
|
 Previous Article | Table of Contents | Next Article 
Deficiency of plasma plasminogen activator inhibitor 1 results in
hyperfibrinolytic bleeding
MH Lee, E Vosburgh, K Anderson and J McDonagh
Department of Pathology, Beth Israel Hospital, Boston, MA 02215.
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.
Volume 81,
Issue 9,
pp. 2357-2362,
05/01/1993
Copyright © 1993 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. A. Komissarov, A. Zhou, and P. J. Declerck
Modulation of Serpin Reaction through Stabilization of Transient Intermediate by Ligands Bound to {alpha}-Helix F
J. Biol. Chem.,
September 7, 2007;
282(36):
26306 - 26315.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
E. Duggan, M. J. O'Dwyer, E. Caraher, D. Diviney, E. McGovern, D. Kelleher, R. McManus, and T. Ryan
Coagulopathy After Cardiac Surgery May Be Influenced by a Functional Plasminogen Activator Inhibitor Polymorphism
Anesth. Analg.,
June 1, 2007;
104(6):
1343 - 1347.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Repine and M. Osswald
Menorrhagia Due to a Qualitative Deficiency of Plasminogen Activator Inhibitor-1: Case Report and Literature Review
Clinical and Applied Thrombosis/Hemostasis,
July 1, 2004;
10(3):
293 - 296.
[Abstract]
[PDF]
|
|
|
|
|
|
|
T. Kietzmann, U. Roth, and K. Jungermann
Induction of the Plasminogen Activator Inhibitor-1 Gene Expression by Mild Hypoxia Via a Hypoxia Response Element Binding the Hypoxia-Inducible Factor-1 in Rat Hepatocytes
Blood,
December 15, 1999;
94(12):
4177 - 4185.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
N. Nagai, T. Urano, Y. Takada, and A. Takada
Reperfusion After Venous Occlusion Caused Transient Increase in Plasminogen Activator Inhibitor-1 in Systemic Circulation
Clinical and Applied Thrombosis/Hemostasis,
April 1, 1998;
4(2):
133 - 137.
[Abstract]
[PDF]
|
|
|
|
|
|
|
W. P. Fay, A. C. Parker, L. R. Condrey, and A. D. Shapiro
Human Plasminogen Activator Inhibitor-1 (PAI-1) Deficiency: Characterization of a Large Kindred With a Null Mutation in the PAI-1 Gene
Blood,
July 1, 1997;
90(1):
204 - 208.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
G. Christ, P. Hufnagl, C. Kaun, G. Mundigler, G. Laufer, K. Huber, J. Wojta, and B. R. Binder
Antifibrinolytic Properties of the Vascular Wall: Dependence on the History of Smooth Muscle Cell Doublings In Vitro and In Vivo
Arterioscler Thromb Vasc Biol,
April 1, 1997;
17(4):
723 - 730.
[Abstract]
[Full Text]
|
|
|
|
|
|
|
I. M. Lang, T. L. Chuang, C. F. Barbas III, and R. R. Schleef
Purification of Storage Granule Protein-23. A NOVEL PROTEIN IDENTIFIED BY PHAGE DISPLAY TECHNOLOGY AND INTERACTION WITH TYPE I PLASMINOGEN ACTIVATOR INHIBITOR
J. Biol. Chem.,
November 22, 1996;
271(47):
30126 - 30135.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
I. M. Lang and R. R. Schleef
Calcium-dependent Stabilization of Type I Plasminogen Activator Inhibitor within Platelet alpha-Granules
J. Biol. Chem.,
February 2, 1996;
271(5):
2754 - 2761.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
