[Pattern of type A hemophilia in Senegal: prospective study in 54 patients].

Little is known about hemophilia in the developing countries because of the difficulties in the diagnosis and the therapeutical management of this disease. Here we present the results of the follow-up of 54 patients in Senegal. Diagnosis was always confirmed by measuring the biological activity of factors VIII and IX. Patients were treated at home or in the hematology service according to the gravity of hemorrhage events. The severe form represented 29.6%, moderate form was 55.6% and minor form 14.8%. Total number of hemorrhage events was 1078 per year: 449 hemarthrosis (41.7%), 373 exteriorized hemorrhage (34.7%) and 256 hematomas (23.7%). Mean frequency of hemarthrosis per patient per year was 12 in severe form, 8 in moderate form and 3 in minor form. Mean frequency of hematomas per patient per year was 5.2 in severe, 4.9 in moderate and 4.2 in minor form. For exteriorized hemorrhage, the mean frequency was 7.06 in severe, 7.4 in moderate and 6.5 in minor form. The severity of hemophilia significantly influenced the frequency of hemarthrosis (P = 0.02) but not the frequency of hematoma (P = 0.6) and exteriorized hemorrhage (P = 0.6). Treatment of these accidents was performed at home (88.5%), in day hospital (9.5%) or needed hospitalization (1.8%). Three patients have died during this three-year survey, one because of HIV infection and the two others from digestive hemorrhage. In conclusion, lesser morbidity and mortality were observed when compared with previously. The importance of a regular follow up and education of patients must be emphasized especially if factor concentrates are not available.