Résumé
Patients and methods: Five hundred eighty nine patients whose main symptom was the presence of mucocutaneous hemorrhages were studied. Bleeding time, platelet count, coagulant activity of factor VIII (FVIII:C), FvW: Ag and FvW:CoRis and ABO blood group were measured in all patients in a first stage. According to the results of these tests, further studies were decided. Results: In patients younger than 13 years old, males predominated and, in older patients, females consulted with higher frequency. There was a higher proportion of individuals with O blood type than in the normal population. Bleeding time was abnormal in 330 patients (56 percent). One hundred ten patients (19 percent) had von Willebrand disease and, among them, one third had a normal bleeding time. Isolated reduction of factor VIII activity was found in 66 patients (11 percent, 51 males) and 32 of these had normal bleeding time. Eighty one patients (14 percent) were considered to have an hereditary platelet function defect. A precise diagnosis was not achieved in 332 patients (56 percent). Conclusions: Among patients consulting for mucocutaneous hemorrhages, 19 percent had von Willebrand disease, 11 had an isolated reduction of factor VIII activity, 14 percent had platelet function defects and in 56 percent, a precise diagnosis was not reached