PB1132 - The new era of Haemophilia in a Comprehensive Hemophilia Treatment Center in Mexico

Background: Haemophilia is a chronic condition, that requires specialized treatment. It´s recommended that the People with Haemophilia (PwH) be managed in a Comprehensive Hemophilia Center (CHC).

Aims: The aim of this study is to assess the current haemophilia situation in our CHC.

Methods: Electronic records from the last 2 y. of PwH were reviewed at the CIHT (Centro Integral en Hemostasia y Trombosis) in Regional Hospital No. 1, in Mexico City, Mexico

Results: The total population of PwH in our center is 120 patients; of which Haemophilia A (HA) are 103 (85.9%) & Haemophilia B (HB) 17 patients (14.1%). The median (range) age was 31 (1-86) years; the pediatric population is 17, and PwH and inhibitors are 9 (8.7%), all of them with HA and high response inhibitors. 80% of the total have arthropathy, of which those with HA are 81 (78.5%), and with HB are 15 (88.2%). 21 patients have >45 years old and 62% of the them have comorbidities (Graph 2). Transfusion related infections were present in 27 PwH.
Treatment regimen: Most patients with severe hemophilia were treated with prophylaxis 76/82 (93%). All children with severe haemophilia received prophylaxis; and 8/11 children had arthropathy. All patients with HA received rFVIII-SHL (recombinant FVIII of Standard Half-Life), and pdFIX (plasma-derived FIX) for HB. The frequency of prophylaxis is according with the guidelines. 8/9 patients with HA and high response inhibitors are treated with prophylaxis with emicizumab. Only 1 patient is treated with ITI.

Conclusion(s): In this new era where Clotting Factor Concentrates are a reality in our center, all patients with severe haemophilia should be managed with a strategy of bleeding prevention and we need to focus on the prevention of arthropathy. Indeed is essential the comprehensive treatment for the PwH.