PB1205 - Joint health in people with hemophilia A and inhibitors during emicizumab prophylaxis (EMCase Study)

Tuesday, June 25, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Ricardo Mesquita Camelo, MD, MBA, MSc, PhD, PostD

Professor
Department of Internal Medicine, Faculty of Medicine, Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil
Belo Horizonte, Minas Gerais, Brazil

Co-Author(s)

LH

Luila Clicia Moura Henriques

Student
Faculty of Pharmacy, Universidade Federal de Minas Gerais (UFMG)
Belo Horizonte, Minas Gerais, Brazil
CF

Clarissa Barros Ferreira

Hematologist
Centro de Hematologia e Hemoterapia do Rio Grande do Sul (HEMORGS)
Porto Alegre, Rio Grande do Sul, Brazil
AL

Adriana Celia Luz

Physiotherapist
Centro de Hematologia e Hemoterapia do Rio Grande do Sul (HEMORGS)
Porto Alegre, Rio Grande do Sul, Brazil
LC

Luany Elvira Mesquita Carvalho

Hematologist
Centro de Hematologia e Hemoterapia do Ceará (HEMOCE)
Fortaleza, Ceara, Brazil
TR

Tatyane Oliveira Rebouças

Hematology Nurse
Centro de Hematologia e Hemoterapia do Ceará (HEMOCE)
Fortaleza, Ceara, Brazil
MS

Melissa Palis Santana

Hematologist
Centro de Hematologia e Hemoterapia do Piauí (HEMOPI)
Teresina, Piaui, Brazil
SF

Sandra Sibele Figueiredo

Hematologist
Centro de Hematologia e Hemoterapia da Paraíba (HEMOÍBA)
João Pessoa, Paraiba, Brazil
EC

Edilma Silva Casaretto

Hematology Nurse
Centro de Hematologia e Hemoterapia da Paraíba (HEMOÍBA)
João Pessoa, Paraiba, Brazil
JA

Juliana Alvares-Teodoro

Professor of Pharmacy
Faculty of Pharmacy, Universidade Federal de Minas Gerais (UFMG)
Belo Horizonte, Minas Gerais, Brazil

Background: Recurrent hemarthroses can result in arthropathy in people with hemophilia A (PwHA). Prophylaxis aims to reduce bleeding events and prevent the onset or progression of hemophilic arthropathy. Although it is recognized that emicizumab prophylaxis reduces hemarthroses frequency, little is known about its impact on joint health.

Aims: We aimed to evaluate joint health in PwHA on emicizumab prophylaxis.

Methods: PwHA on emicizumab prophylaxis for at least 1 year were invited at several Brazilian Hemophilia Treatment Centers. Clinical and treatment data before (12 months) and during (12 months) emicizumab prophylaxis were collected. Bleeds were classified according to their cause (spontaneous, post-traumatic and unknown), and only counted if any hemostatic treatment was applied. Joint health was assessed using the Haemophilia Joint Health Score (HJHS) for each joint and total. Higher scores correspond to worse joint health.

Results: Of a total of 13 PwHA included, 9/13 (69%) had severe hemophilia A and 13/13 (100%) failed immunotolerance and had inhibitors receiving bypass agents. Before starting emicizumab, 10/13 (77%) were on prophylaxis. The median age at emicizumab prophylaxis start was 14 years (range 6-71). The total number of treated bleeding events reduced from 37 (17 spontaneous) to 2 (1 spontaneous and 1 post-traumatic), before and during emicizumab prophylaxis, respectively. Comparing these periods, the median (range) annual bleeding rate reduced from 1 (0-12) to 0 (0-1) and the number of PwHA with zero bleed increased from 3/13 (23%) to 11/13 (85%). The total number of affected joints (HJHS > 0) slightly increased from 39 before to 40 during emicizumab prophylaxis, with the left ankle being the most affected joint at both periods. The medians (range) of the HJHS-total score were 9 (0-34) and 11 (0-33), before and during prophylaxis with emicizumab, respectively.

Conclusion(s): Baseline joint health in this small population did not change after 1-year emicizumab prophylaxis.