OC 60.4 - Joint damage in patients affected by Von Willebrand disease: a single-center experience

Tuesday, June 27, 2023

15:30 – 15:45 ET

Room: 516

Session Available As: Live-Stream and On-Demand

Presenting Author(s)

Roberta Gualtierotti, MD, PhD

Associate Professor
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Centro Emofilia e Trombosi Angelo Bianchi Bonomi
Milan, Lombardia, Italy

Co-Author(s)

Sara Arcudi, MD

Academic researcher, MD, PhD
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Centro Emofilia e Trombosi Angelo Bianchi Bonomi
Milan, Lombardia, Italy
AG

Andrea Giachi

Internal Medicine Resident
Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milan, Lombardia, Italy
MS

Margherita Smania

Hematology Resident
Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milan, Lombardia, Italy
Addolorata Truma, MD

Resident doctor in Internal Medicine
Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milano, Lombardia, Italy
Alessandro Ciavarella, MD (he/him/his)

Specialized Doctor
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Centro Emofilia e Trombosi Angelo Bianchi Bonomi
Milan, Lombardia, Italy
VB

Valentina Begnozzi

Physiotherapist
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
EB

Elena Boccalandro

Physiotherapist
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Eugenia Biguzzi, MD

Hematologist
Hematology clinic, Oncology Institute of Southern Switzerland, EOC
Bellinzona, Ticino, Switzerland
SS

Simona Maria Siboni

Hematologist
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center
Milan, Lombardia, Italy
Flora Peyvandi, MD, PhD (she/her/hers)

Director
IRCCS Ca' Granda Maggiore Hospital Foundation, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milano, Italy; Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milano, Italy
Milano, Lombardia, Italy

Background: Von Willebrand Disease (VWD) is caused by a quantitative or qualitative defect of von Willebrand factor (VWF), implying a variable degree of FVIII deficiency. Patients with FVIII levels < 5% can develop an arthropathy comparable to moderate/severe hemophilia A. Joint ultrasound is a valid tool to detect and quantify arthropathy in patients affected by hemophilia, but data on VWD are scarce.

Aims: Our aim was to determine the joint health status of patients affected by VWD using clinical examination and joint ultrasound scores and to correlate them with FVIII levels.

Methods: Patients with VWD (group 1 FVIII < 20%; group 2 FVIII 20-40%; group 3 FVIII >40%) followed at the Angelo Bianchi Bonomi Hemophilia and Thrombosis Center in Milan, Italy were enrolled. Joint status was evaluated by physical examination using the Hemophilia Joint Health Score 2.1 (HJHS) and by ultrasound using the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score. Spearman's rho was used to evaluate correlation between two variables. * p< 0.005; **p < 0.001

Results: Forty-six patients were included, of which 17 for group 1, 14 for group 2, 15 for group 3, mean age (±standard deviation - SD) 38±19 years, 54% females, with a mean±SD HJHS total score of 4.57±7.3 and a HEAD-US total score of 4.4±6.5. We found a strong correlation between HJHS vs HEAD-US 0.70**. FVIII levels at diagnosis were significantly inversely correlated with both HJHS -0.37* and HEAD-US -0.48**. No significant correlation was found between age and both HJHS and HEAD-US

Conclusion(s): We report a strong correlation between the physical examination and joint ultrasound scores of joint health status in patients with VWD and a significant correlation between joint health status and patient FVIII levels at diagnosis.
Acknowledgments: this study was financially supported by the Grifols Martin Villar Haemostasis Award.

Learning Objectives:

Upon completion, participants will be able to recognize the importance of musculoskeletal examination in Von Willebrand Disease
Upon completion, participants will recognize the importance of ultrasound evaluation in Von Willebrand Disease
Upon completion, participants will recognize the importance of a multidisciplinary team for the evaluation of musculoskeletal health in patients with Von Willebrand Disease