PB1275 - Multicenter and cross-sectional study of joint damage of patients with non-severe hemophilia: A "Mediterranean Group" study

Tuesday, June 27, 2023

18:30 – 19:30 ET

Room: Exhibition

Presenting Author(s)

Ihosvany Fernández-Bello, PhD (he/him/his)

Postdoctoral Researcher
Hemostasis and Thrombosis Unit, Regional University Hospital of Málaga, IBIMA, Málaga, Spain.
Málaga, Andalucia, Spain

Co-Author(s)

Francisco José López Jaime, MD

Hematologist
Hemostasis and Thrombosis Unit, Regional University Hospital of Málaga, IBIMA, Málaga, Spain.
Málaga, Andalucia, Spain
Jose Manuel Calvo Villas, MD (he/him/his)

Hematology Department
HOSPITAL UNIVERSITARIO MIGUEL SERVET ZARAGOZA
Madrid, Madrid, Spain
AM

Ana Marco

Hematologist
University General Hospital Dr. Balmis, Alicante, Spain
Alicante, Comunidad Valenciana, Spain
SH

Sonia Herrero Martín

Hematologist
University Hospital of Guadalajara
Almeria, Andalucia, Spain
LE

Laura Entrena

Hematology Deparment
Hospital Universitario Virgen de las Nieves
Granada, Andalucia, Spain
AM

Adrián Montaño, PhD (he/him/his)

Postdoctoral researcher
Hemostasis and Thrombosis Unit, Regional University Hospital of Málaga / University of Salamanca
Málaga, Andalucia, Spain
BD

Bolívar L- Díaz Jordán

Hematologist
General Hospital of Valdepeñas
Valdepeñas, Castilla-La Mancha, Spain
CG

Covadonga García Díaz

Haematology Department
Hospital Universitario de Burgos
Burgos, Castilla y Leon, Spain
SJ

Sergio Jurado Herrera

Hematologist
Torrecárdenas University Hospital
Torrecardenas, Andalucia, Spain
NP

Noelia Pérez González

Hematologist
Torrecárdenas University Hospital
Torrecárdenas, Andalucia, Spain
MC

Mariana Canaro

Hematologist
Son Espases University Hospital
Palma de Mallorca, Islas Baleares, Spain
FG

Faustino García Candel

Hematology Department
Hospital Clínico Universitario Virgen de la Arrixaca (Murcia)
Murcia, Murcia, Spain
PM

Pascual Marco

Hematologist
Department of Clinical Medicine, Universidad Miguel Hernández, Alicante, Spain
Alicante, Comunidad Valenciana, Spain

Background: Patients with non-severe hemophilia (nSH) might present joint damage (JD) but data are scarce. In nSH, age and factor (F) VIII/FIX basal levels might influence JD but the correspondence between JD and FVIII/FIX basal levels, age and global hemostatic capacity (GHC) has been poorly studied.

Aims: We aim to evaluate the influence of FVIII/FIX basal levels, age and GHC on the JD of patients with nSH.

Methods: Cross-sectional/prospective study in 10 Spanish hospitals was performed. nSH patients ≥12 years old without history of prophylactic treatment were included. Number of patients with JD (HEAD-US>0), basal levels of FVIII-CSA (chromogenic assay) and FVIII/FIX-OSA (clotting assay), fibrinogen (Fib), thrombin generation test (TGT) (Genesia), thromboelastometry (ROTEM), platelet (PTS) and erythrocyte (ERY) counts were analyzed. Lineal regression analysis using HEAD-US as dependent variable were evaluated.

Results: 83 nSH patients (9 moderate and 74 mild) were included. PTS, ERY and Fib were normal. HEAD-US score was evaluated in 59 patients (9 moderate [MoH] and 50 mild [MiH]; 354 joints). 75% of MoH and 54% of MiH had JD (HEAD-US>0). Median JD was similar between MoH and MiH (5.0 [2.0-8.5] in MiH and 4.0 (0.25-8.8) in MoH; p= 0.5015). FVIII/FIX was similar between the group with JD: 17.0 (11.0-34.0) IU/dL and without JD: 15.5 (11.0-22.0) IU/dL; p= 0.4864 (statistical power> 80%). 7% of MiH presented at least one target joint. Neither age, FVIII/FIX baseline levels nor GHC were predictive of the HEAD-US score.

Conclusion(s): JD might be similar between patients with nSH. Correlation between FVIII/FIX and GHC was detected. MiH patients might develop target joints. Further studies are needed to assess the influence of FVIII/FIX basal levels, age and CHG on the JD. Our results highlight the importance of evaluating JD and QoL in nSH in order to clarify if new protocols for JD diagnosis, prevention, and treatment are required.