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Thromb Haemost 1990; 63(01): 036-038
DOI: 10.1055/s-0038-1645682
Original Article
Schattauer GmbH Stuttgart

The Effect of Combined Factor XI Deficiency with von Willebrand Factor Abnormalities on Haemorrhagic Diathesis

Shulamith Tavori
The Coagulation Laboratory, Hematology Institute, Rambam Medical Center and Faculty of Medicine, Technion-lsrael Institute of Technology, Haifa, Israel
,
Benjamin Brenner
The Coagulation Laboratory, Hematology Institute, Rambam Medical Center and Faculty of Medicine, Technion-lsrael Institute of Technology, Haifa, Israel
,
llana Tatarsky
The Coagulation Laboratory, Hematology Institute, Rambam Medical Center and Faculty of Medicine, Technion-lsrael Institute of Technology, Haifa, Israel
› Author Affiliations
Further Information

Publication History

Received 21 December 1989

Accepted after revision 17 October 1989

Publication Date:
02 July 2018 (online)

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Summary

To account for the lack of correlation between the level of factor XI (FXI) in deficient patients and haemorrhagic manifestations, we correlated the prevalence of combined FXI and von Willebrand’s factor (vWF) deficiency in 212 FXI-deficient patients. Fifty-four patients had a combined FXI and vWF deficiency: 16 patients had severe and 38 patients had mild FXT deficiency. In a group of 28 patients with comparably mild FXI deficiency, 14 bleeders had significantly lower mean vWF, Ag, ristocetin cofactor and ristocetin induced platelet aggregation than 14 non-bleeders selected on the basis of comparable FXI levels. These findings suggest that the combination of FXI and vWF deficiency is common and may affect the bleeding tendency in mild FXI deficiency.

Keywords

FXI-deficiency - vWF - Combined factor deficiency
 

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