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Thromb Haemost 1984; 51(03): 371-375
DOI: 10.1055/s-0038-1661104
Original Article
Schattauer GmbH Stuttgart

Acquired Factor XI Inhibitors in Two Patients with Hereditary Factor XI Deficiency

Kangathevy Morgan
The Departments of Medicine and Biochemistry, University of Southern California, School of Medicine, Los Angeles, California, U.S.A.
,
Sandra Schiffman
The Departments of Medicine and Biochemistry, University of Southern California, School of Medicine, Los Angeles, California, U.S.A.
,
Donald Feinstein
The Departments of Medicine and Biochemistry, University of Southern California, School of Medicine, Los Angeles, California, U.S.A.
› Author Affiliations
Further Information

Publication History

Received 28 December 1983

Accepted 06 April 1984

Publication Date:
19 July 2018 (online)

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Summary

Two patients with hereditary factor XI deficiency developed inhibitors following plasma transfusions. Neither had severe spontaneous bleeding. The patients’ plasmas neutralized both factor XI in plasma, purified factor XI, and purified factor XIa. The inhibitor in both patients’ plasmas adsorbed to Protein A- Sepharose. The inhibitors eluted from Protein A-Sepharose were partially neutralized by kappa and lambda light chain antisera indicating that they were polyclonal IgG antibodies. Both inhibitors markedly decreased adsorption of factor XI to glass surfaces. The cleavage of factor XI by trypsin was unaffected by the inhibitors. The lack of severe spontaneous bleeding in both of these patients strongly suggests that an alternate coagulation mechanism bypassing factor XI must compensate for this severe defect.

Keywords

Factor XI inhibitors - Factor XI deficiency
 

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