Life-threatening bleeding in patients with hemophilia (PWH): a 10-year cohort study in Dakar, Senegal

Abstract

Life-threatening bleeding (LTB) is one of the major complications of hemophilia with a high risk of mortality. Objective: The aim of this study was to assess incidence, risk factors, treatment and outcome of LTB in Senegalese people with hemophilia (PWH). Methods: We analyzed the characteristics of LTB in a cohort of 274 PWH after 10 years of follow-up. Results: We included 274 patients (241 HA and 33 HB). The mean age was 16.45 years and the median age was 13 years. The mean annual bleeding rate (ABR) was 1.65 (2.83 for severe form, 1.54 for moderate form, and 1.22 for mild form). A replacement therapy with clotting factor concentrates (CFC) was administered to 217 patients (79.2%); 56 patients (20.4%) received low-dose prophylaxis (LDP). Prevalence of inhibitors was 4.7% (13/274). All patients were HIV and HCV antibody negative. We observed 31 cases of LTB in 22 patients with an incidence of 8.03%. Central nervous system (CNS) bleeds were most frequent (6.2%) and accounted for 54.8% of severe bleeding. The delay between the first signs and the emergency visit was 78.9 hours. Inhibitors were positive in one patient among those who presented LTB. These bleeding were treated with CFC in 16 patients, surgical drainage (1 patient) and electrocoagulation during gastroscopy (1 patient). Eleven patients had complete remission and two had sequelae. We reported 0.32 death per 100 person-years. CNS bleeds were the main cause (77.7%). Four patients were secondarily on LDP. We observed a significant correlation between treatment (after 2 hours) and mortality. Conclusion: LTB is a serious and lethal complication in PWH in absence of early management. A good awareness of patients and their family would further reduce this incidence, especially in resources-limited countries.