We present here the first descriptive analysis of the French cohort of patients with inherited coagulation protein deficiencies, the result of close collaboration between healthcare professionals and the coordinating center located within the Department of Chronic Diseases and Trauma at the French Institute for Public Health Surveillance. As of the date of the analysis (October 8, 2005), 4,018 patients from 38 treatment centers had been included. The diagnoses were hemophilia A (n=2,901, including 1,306 severe cases), hemophilia B (n=605, including 229 severe cases), von Willebrand disease (n=375), and other coagulation protein deficiencies (n=137). A major recruitment effort was undertaken, with nearly 3,000 new patients enrolled in the last two years, although the registry has not yet achieved full coverage of French patients (approximately 6,000 patients expected). This analysis reviews several health outcomes. Viral infections, particularly those caused by the hepatitis C virus and the human immunodeficiency virus (HIV), mortality, and the emergence of an inhibitor for one of the coagulation factors were analyzed, as well as patient management based on the type of deficiency and its severity. This report outlines several areas of focus for 2006: - improving patient recruitment; - more systematically exploiting the knowledge potential of this database regarding the PUPS subcohort, the determinants of inhibitor development, the prophylactic use of coagulation factors, and the causes of patient mortality. (R.A)