Factor XI deficiency in Iranians: its clinical manifestations in comparison with those of classic hemophilia.
OBJECTIVE
In patients with factor XI(FXI) deficiency the bleeding tendency is poorly correlated with plasma factor levels. The purpose of this study was to evaluate whether or not this discrepancy is also present in a large series of patients from Iran, a previously unexplored ethnic group.


METHODS
In 28 FXI - deficient patients bleeding symptoms and their relation to FXI levels were compared with those of 100 patients with factor VIII (FVIII)deficiency (classic hemophilia), matched for severity of factor deficiency.


RESULTS
Spontaneous bleeding was definitely less frequentin FXI deficiency than in hemophilia, whereas postoperative and post-traumatic bleeding occurred with comparable frequencies. Among FXI-deficient patients the severity of symptoms was poorly correlated with FXI levels, mildly deficient patients bleeding almost as frequently as those severely deficient. In contrast, in patients with classic hemophilia there was a close relation between the severity of bleeding and degree of FVIII deficiency.


CONCLUSIONS
As in other ethnic groups, in Iranians factor XI deficiency is less severe than classic hemophilia and the bleeding tendency is poorly correlated to plasma factor levels.
Peyvandi
Flora
F
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, IRCCS Maggiore Hospital and University of Milan, Italy.
Lak
Manijeh
M
Mannucci
Pier Mannuccio
PM
D005172:Factor XI / Q000032:analysis; D005173:Factor XI Deficiency / Q000453:epidemiology* / Q000473:pathology; D006467:Hemophilia A / Q000453:epidemiology / Q000473:pathology; D006470:Hemorrhage / Q000097:blood / Q000453:epidemiology / Q000209:etiology; D006801:Humans; D007492:Iran / Q000453:epidemiology
D005172:Factor XI