FranceCoag Network: A National Multicenter Prospective Cohort for Congenital Bleeding Disorders.

The FranceCoag Network is a prospective national cohort of patients affected with hemophilia and severe hereditary hemorrhagic diseases except platelet disorders. It has been set up in France since January 2003 as replacement of a previous project only dedicated to hemophilia. The objectives are to know the exhaustive number, type and characteristics of these patients registered in hemophilia centers. Inclusion criteria are: FVIII or IX defect (<30%), severe defect (<10%) in FII, V, VII, X, XI, XIII or FI (< 0.1 g/l) or severe VWD. Once per year centers send datas on severe bleeding episodes, surgery, replacement therapy i.e. type of concentrate, units, place of treatment and main complications (including new inhibitors). These data are collected and monitored by a coordinating center attached to a public health institution (InVS). Direct transmission of datas via a highly protected web site is foreseen for the end of 2005. The follow-up of the study is ensured by a steering committee (regional coordinators, clinicians, committed health institutions, representatives of patients association) periodically extended to scientific experts and other public health agencies. Beside its epidemiologic interest, this cohort will also promote research projects on this population such as Pups cohort (defined as severe hemophilia A or B patients with none or few cumulative exposure days at the enrolment). On July 2005, 3469 patients were included by 37 centers. The diagnosis are hemophilia A n=2594 (75%); hemophilia B n=539 (15%), VWD n=239 (7%), severe defect in other coagulation factor: n= 97 (3%). At their inclusion, 373 patients (11%) were infected by HIV1, 1383 (40%) had a positive HCV serology, while 365 patients (10%) were co infected by HIV1 and HCV. Among this cohort, 45 deaths were observed since 1994, which represent 4.5 deaths per 1 000 years-patients (95% CI: [3.3–6.0]). Deaths were related to AIDS or HCV in 19 cases. Inhibitors was observed in 388 patients (11%) (hemophilia A: n=369, hemophilia B n=15, VWD n=2, FXIII defect n= 1, FV defect n=1). The incidence of inhibitor during the follow-up was 9.4 cases per 1000 person-years.