PB0245 - Ultrasound for the assessment of arthropathy in patients with moderate hemophilia A: a multi-center cross-sectional study.

Sunday, June 23, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

IC

Ilenia Calcaterra

Assistant Professor
Federico II University of Naples
Napoli, Campania, Italy

Co-Author(s)

FV

Federica Valeri

Physician
Regional Centre for Hemorrhagic and Thrombotic Diseases, AOU molinette10126 Turin, Italy
Torino, Piemonte, Italy
EB

Erminia Baldacci

Physician
Haematology, “Umberto I” Policlinico, Department of Translational and Precision Medicine, Sapienza University of Rome, 00118 Rome, Italy
Roma, Lazio, Italy
MN

Marisanta Napolitano

Professor
University of Palermo
Palermo, Sicilia, Italy
EZ

Ezio Zanon

Physician
Haemophilia Centre, General Medicine, Padua University Hospital, 35121 Padua, Italy
Padova, Veneto, Italy
Gm

Gabriella mazucconi

Physician
University Hospital, Ferrara, Italy;
Ferrara, Emilia-Romagna, Italy
CG

Cornelia Guerrino

Pharmacist
University Federico II of Naples
Naples, Campania, Italy
SD

Sofia Donnarumma

Resident
University Federico II of Naples
Naples, Campania, Italy
VP

Vincenzina Palermo

Resident
University Federico II of Naples
Naples, Campania, Italy
EC

Ernesto Cimino

assistant professor
DEPARTMENT OF CLINICAL MEDICINE AND SURGERY. Federico II University of Naples
Naples, Campania, Italy
SS

Sergio Siragusa

Professor
University of Palermo
Palermo, Sicilia, Italy
CM

Carlo Martinoli

Professor
DISSAL - Università di Genova, Via A. Pastore 1, I-16132 Genova, Italy.
genova, Liguria, Italy
MD

Matteo Di Minno

Professor
Department of Clinical Medicine and Surgery, Federico II University of Naples, Naples, Italy
Naples, Campania, Italy

Background: The risk of developing haemophilic arthropathy (HA) in patients with moderate haemophilia A (moHemA) is highly variable. People with moHemA seem to be under-treated, and this may lead to joint damage and worsen quality of life.

Aims: The aims of the present study were: i) to evaluate the added value of ultrasound (US) compared to clinical examination in identifying moderate HA patients at high risk of developing HA; ii) to investigate the potential of the HEAD-US in driving treatment modifications in these patients.

Methods: We performed multicenter cross-sectional study. Consecutive patients with moHemA receiving on-demand replacement treatment underwent a clinical assessment of joint status for all the six major synovial joints according to HJHS protocol. On the same day, all patients underwent an US examination performed according to the HEAD-US protocol.

Results: A total of 51 subjects were included and 306 joints were evaluated by clinical and US examination. The median HJHS value was 2.0(IQR: 0-3), with a score ranging from 0 to 1 found in 25 patients (49.0%) and a score > 4 in 6 (11.8%). The HEAD-US median value was 2.0(IQR: 1-7) and a statistically significant correlation between HJHS and HEAD-US was found (rho=0.732, p< 0.001). HEAD-US was concordant with HJHS in the identification of the 6 patients with arthropathy, but it also detected pathological joint changes in 8% of patients with a HJHS ranging from 0 to 1. Overall, a pathologic HEAD-US was found in 16 patients with moderate haemophilia (31.4%).

Conclusion(s): Our study suggests that the prevalence of HA in patients with moHemA receiving on-demand treatment is not negligible (≈30%) and that US able to detect clinically overt as well as sub-clinical changes in this clinical setting.