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Thromb Haemost 1999; 81(02): 203-207
DOI: 10.1055/s-0037-1614443
Review Articles
Schattauer GmbH

Low Levels of Heparin-releasable Tissue Factor Pathway Inhibitor in Young Patients with Thrombosis

R. A. S. Ariëns
1   From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Ospedale Maggiore IRCCS and University of Milan, Italy
,
G. Alberio
1   From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Ospedale Maggiore IRCCS and University of Milan, Italy
,
M. Moia
1   From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Ospedale Maggiore IRCCS and University of Milan, Italy
,
P. M. Mannucci
1   From the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Ospedale Maggiore IRCCS and University of Milan, Italy
› Author Affiliations
Further Information

Publication History

Received08 May 1998

Accepted after resubmission26 October 1998

Publication Date:
08 December 2017 (online)

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Summary

An association between deficiency of tissue factor pathway inhibitor (TFPI) and thrombosis has not been clearly demonstrated in humans, but previous studies have focused on the measurement of plasma TFPI, which is only a small part of the total body TFPI. The major fraction of this natural anticoagulant can be measured in plasma after release by heparin injection. To investigate if deficiency of heparin-releasable TFPI is associated with thrombosis, we measured TFPI activity in plasma before and 10 min after intravenous injection of 7500 IU unfractionated heparin in 64 young patients with venous thrombosis, 49 young patients with arterial thrombosis and 38 healthy individuals. Post-heparin TFPI activity levels were significantly lower in the group of patients with venous thrombosis than in controls (mean ± SD: 230% ± 39 vs 260% ± 34, p = 0.0002), whereas there was no difference for patients with arterial thrombosis. Defining the normal range as the mean ± 2 SD of TFPI activity in controls, twelve patients had low post-heparin TFPI activity levels, seven with venous and five with arterial thrombosis. Low levels of TFPI activity were confirmed by immunoassay in six of the seven patients with venous thrombosis and two of the five patients with arterial thrombosis, and were present also in at least one first degree relative of six patients, suggesting that the defect might be inheritable. However, the causative role of low heparin-releasable TFPI remains uncertain, because co-segregation of the defect with thrombotic symptoms could not be demonstrated in the small number of families studied.

 

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