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Thromb Haemost 1988; 59(03): 468-473
DOI: 10.1055/s-0038-1647517
Original Article
Schattauer GmbH Stuttgart

Antithrombin Oslo: Type Ib Classification of the First Reported Antithrombin-Deficient Family, with a Review of Hereditary Antithrombin Variants

Mae B Hultin
*   The Division of Hematology, Dept. of Medicine, State University of New York at Stony Brook, the Veterans Administration Medical Center, Northport, NY, USA
,
John McKay
**   The Immunology Dept., Aukland Hospital Aukland, New Zealand
,
Ulrich Abildgaard
***   The Medicine Dept. A, Aker Hospital, Oslo, Norway
› Author Affiliations
Further Information

Publication History

Received 15 June 1987

Accepted after revision 19 February 1988

Publication Date:
29 June 2018 (online)

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Summary

Patients with classical antithrombin deficiency (Type I) from seven unrelated kindreds were studied by crossed immunoelectrophoresis of plasma in the presence and absence of heparin. The only abnormal pattern was found in the kindred first reported by Egeberg in 1965. An abnormal cathodal peak of antithrombin antigen was found in the presence, but not the absence, of heparin in the first dimension gel. We have named this variant antithrombin Oslo. Such evidence of an abnormal protein, despite equivalent low levels of antithrombin antigen and activity, has been denoted previously by Sas as Type Ib deficiency. In the context of this new report, we review the literature to date on 33 other variants of the Types Ib, II and III subclassifications with a discussion of the value of the classification scheme.

Keywords

Antithrombin III - Familial antithrombin deficiency - Antithrombin antigen
 

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