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KA Bauer, JB Ashenhurst, J Chediak and RD Rosenberg
A family with a high incidence of spontaneous thromboembolism over four
generations has been investigated. The propositus is a 21-yr-old male with
a history of thrombophlebitis. Medical histories of 46 family members were
obtained. Twelve of these individuals have experienced deep venous
thromboses and/or pulmonary emboli. Seven members of the kindred, with a
prior history of thrombotic phenomena, were investigated in detail. These
subjects were found to have normal plasma concentrations of immunoreactive
antithrombin (mean 96%), decreased plasma levels of progressive
antithrombin activity (mean 50%), and greatly reduced amounts of plasma
heparin cofactor activity (mean 42%). The abnormal antithrombin ("Chicago")
was found to elute from heparin- Sepharose at a higher ionic strength than
normal inhibitor. The functionally defective antithrombin molecules exhibit
a reduced ability to neutralize thrombin in the presence or absence of
heparin (approximately 10%-20% of normal). The molecular defect of this
protease inhibitor thus appears to be distinct from those of previously
described abnormal antithrombins.
This article has been cited by other articles:
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| Copyright © 1983 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||
