PB0347 - Clinical and Laboratory Characteristics of Patients with Acquired von Willebrand Syndrome

Sunday, June 23, 2024

13:45 – 14:45 ICT

Room: Exhibition Hall

Presenting Author(s)

Alessandro Ciavarella

Clinician
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy

Co-Author(s)

Omid Seidizadeh, MSc, PhD

University of Milan, Department of Pathophysiology and Transplantation, Milan, Italy
Milan, Lombardia, Italy
Luciano Baronciani

Biologist
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Maria Teresa Pagliari

Biologist
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
GC

Giovanna Cozzi

Biologist
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
PC

Paola Colpani

Biologist
Fondazione IRCCS Ca’Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
SA

Sara Arcudi

Clinician
Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy.
Milan, Lombardia, Italy
Roberta Gualtierotti, PhD

Associate Professor
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy
Milan, Lombardia, Italy
CM

Claudia Mistretta

Clinician
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center.
Milan, Alabama, United States
SS

Simona Maria Siboni

Physician
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center
Milan, Lombardia, Italy
Flora Peyvandi, MD, PhD

Full Professor
Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Università degli Studi di Milano, Department of Pathophysiology and Transplantation
Milan, Lombardia, Italy

Background: Acquired von Willebrand syndrome (AVWS) is a rare but often underestimated bleeding disorder characterized by a highly heterogeneous presentation. AVWS occurs in patients without family history of bleeding disorders and frequently in association with other underlying diseases (i.e. lymphoproliferative, myeloproliferative and cardiovascular disorders).

Aims: To describe clinical and laboratory characteristics of patients affected by AVWS.

Methods: We retrospectively evaluated all patients with confirmed diagnosis of AVWS referred to the A. Bianchi Bonomi Hemophilia Center between Jan 1, 2014 and Dec 31, 2023. Clinical data such as the underlying disease and bleeding symptoms, as witnessed by the results of the International Society on Thrombosis and Haemostasis Bleeding Assessment Tool (ISTH-BAT), were retrieved from medical records. In addition, we collected data on laboratory analysis such as the levels of factor VIII, VWF antigen and RiCof, collagen binding and the ratio between VWF propeptide and antigen. Data were reported as median (interquartile range).

Results: Eighty-two consecutive patients with AVWS were included. The vast majority (92%) had a concomitant hematologic disease (63% lymphoproliferative or myeloproliferative diseases, 27% plasma cell dyscrasia - monoclonal gammopathy of undetermined significance, multiple myeloma or Waldenström macroglobulinemia -, 2% amyloidosis), 4% had valvulopathies, 2 patients had ulcerative colitis, while one patient had prostate cancer. Only three patients (4%) did not have any concomitant disease. Clinical and laboratory data are showed in the table. According to the ISTH-BAT, abnormal bleeding was found in 30% of patients and the median ISTH-BAT was 3 (1, 5).

Conclusion(s): Our findings show slightly reduced activity VWF levels in our cohort of patients with AVWS leading often to a mild clinical presentation. However, patients with MGUS or multiple myeloma may show a more severe phenotype. Different mechanisms are involved in AVWS.