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A lifelong bleeding disorder associated with a deficiency of plasminogen
activator inhibitor type 1
J Dieval, G Nguyen, S Gross, J Delobel and EK Kruithof
Laboratory of Hematology, University Hospital, Amiens, France.
A 36-year-old patient was investigated for a lifelong history of epistaxis
and delayed bleeding after minor surgeries. Deficiencies or abnormalities
of the coagulation system, of platelet function, or of factor XIII and
alpha-2-antiplasmin were excluded. Consistently, however, over a period of
7 years, a high basal euglobulin fibrinolytic activity was observed that
was characterized by a high tissue-type plasminogen activator (t-PA)
activity, normal t-PA antigen, and undetectable plasminogen activator
inhibitor type-1 (PAI-1) antigen and activity. The high specific activity
of t-PA (640,000 IU/mg) and the minimal amounts of t-PA/PAI-1 complexes
detected by fibrin zymography suggest that in this patient all t-PA was
active. This is in striking contrast to normal plasma, where the majority
of t-PA is complexed to PAI-1. Thus, in this patient, a severe deficiency
of PAI-1 is associated with a delayed type bleeding tendency. Our
observation underscores the importance of plasma PAI-1 for the
stabilization of the hemostatic plug.
Volume 77,
Issue 3,
pp. 528-532,
02/01/1991
Copyright © 1991 by The American Society of Hematology
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