Bleeding predictors in factor-XI-deficient patients.
Bleeding in factor-XI-deficient patients is mainly injury-related. Parameters influencing bleeding particularly in patients with minor factor XI deficiency have not been defined. We utilized a logistic regression model to analyze parameters influencing bleeding tendency in subjects from 45 families with factor XI deficiency. Bleeding manifestations were documented in 58% of 26 homozygous or doubly heterozygous factor-XI-deficient patients, in 20% of 46 heterozygous factor-XI-deficient patients and in 9% of 47 family members with a normal factor XI genotype. Odds ratios for bleeding in homozygotes or double heterozygotes were 13 and in heterozygotes 2.6 with 95% confidence intervals of 3.8-45 and 0.8-9.0, respectively. Bleeding correlated negatively with factor XI level (r = -0.36, P = 0.0001) with major factor XI deficiency being a strong predictor of bleeding (P = 0.011). Minor factor XI deficiency and blood group O slightly contributed to bleeding. Although factor VIII and factor XI levels were correlated (r = 0.48, P = 0.0001), levels of factor VIII and von Willebrand factor were not predictors of bleeding. Bleeding was more common following operative procedure involving mucosal membranes (P < 0.01). The designed model enabled prediction of bleeding manifestations with an overall accuracy of 78% and 82% in heterozygotes.
Brenner
B
B
Thrombosis & Hemostasis Unit, Rambam Medical Center, Technion, Haifa, Israel.
Laor
A
A
Lupo
H
H
Zivelin
A
A
Lanir
N
N
Seligsohn
U
U
D016022:Case-Control Studies; D005169:Factor VIII / Q000378:metabolism; D005172:Factor XI / Q000235:genetics / Q000378:metabolism; D005173:Factor XI Deficiency / Q000150:complications*; D005838:Genotype; D006470:Hemorrhage / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D016015:Logistic Models; D011237:Predictive Value of Tests; D012044:Regression Analysis; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D005169:Factor VIII; D005172:Factor XI