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Comparison of bleeding tendency, factor XI coagulant activity, and factor
XI antigen in 25 factor XI-deficient kindreds
MV Ragni, D Sinha, F Seaman, JH Lewis, JA Spero and PN Walsh
The relationship of clinical bleeding tendency and factor XI antigen
(XI:Ag) in factor XI deficiency was studied in 78 members of 25 factor
XI-deficient kindreds. Factor XI:Ag was measured in a competitive
radioimmunoassay, using monospecific, heterologous anti-factor XI antibody.
125I-labeled factor XI, and staphylococcal protein A as the precipitating
agent. Deficiency of factor XI clotting activity (XI:C), less than 0.62
U/mL, occurred in 48 individuals, 22 of whom experienced postoperative or
posttraumatic bleeding: Their mean factor XI:C was 0.21 +/- 0.04 U/mL
(SEM), and factor XI:Ag was 0.23 +/- 0.04 U/mL. The remaining 26 had no
clinical bleeding, many despite surgical challenge: Their mean factor XI:C
was 0.30 +/- 0.04 U/mL, and factor XI:Ag was 0.34 +/- 0.05 U/mL. In all, 13
kindreds had between 1 and 11 members with bleeding; the other 12 had none
with deficient hemostasis. Two heterozygous factor XI-deficient individuals
appeared to be positive for cross-reacting material (CRM+). The slope of
the regression line for factor XI:C and factor XI:Ag data points in the 78
individuals tested did not differ from control, and all points fell within
95% confidence limits derived from control. In conclusion, bleeding
tendency appears to be consistent within a given kindred and is not
determined exclusively by factor XI:C or factor XI:Ag levels.
Volume 65,
Issue 3,
pp. 719-724,
03/01/1985
Copyright © 1985 by The American Society of Hematology
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