Introduction:

severe hemophilia A (SAH) is an hereditary hemorrhagic disease, caused by a factor VIII (FVIII) deficiency of less than 1%. It presents with joint bleeding mainly, which causes a hemophilic arthropathy, which affects its functional independence. The use of tertiary prophylaxis with FVIII offers benefits in adults by decreasing the annual bleeding rate (ABR) and improving functional independence.

Objective:

to determine the percentage of patients who manage to maintain an FVIII level greater than 1%, to know if there is an improvement in functional independence, as well as a decrease in the ABR with the prophylactic regimen used.

Methods:

observational, analytical study. Patients with SAH who were controlled at the Hospital de Clínicas “Dr. Manuel Quintela”, during the year 2020, in prophylaxis with FVIII for 12 months were included. Three samples separated in time for FVIII dosing were obtained and the ABR and functional independence were evaluated in each patient.

Results:

8 patients were analyzed, all presented FVIII higher than 1% at one hour and 24 hours after the administration of FVIII. The bleeding episodes were reduced 4.76 times with the use of Prophylaxis (p = 0.019). Functional independence showed that 5/8 patients improved at least 1 point in the Score.

Conclusions:

tertiary prophylaxis in these patients was beneficial in reducing SAD and improving their functional capacity.

        · resumo em Português | Espanhol     · texto em Espanhol     · Espanhol ( pdf )