VPB0004 - Evaluation of the utility of BAT (ISTH-SSC bleeding assessment tool) questionnaire in the diagnostic of acquired von Willebrand syndrome

Sunday, July 10, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: Virtual

Co-Author(s)

MC

Maria Joana Costa-Pinto

PhD
Servicio de Hematología y Hemoterapia. Unidad de Trombosis y Hemostasia. Complejo Hospitalario Universitario A Coruña.
La Coruña, Galicia, Spain
MF

María Teresa Fernández

MD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain
NR

Nicanor Rodríguez

MD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain
AP

Almudena Pérez-Rodríguez

PhD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain

Presenting Author(s)

MG

María del Carmen Gómez-del-Castillo

MD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain

Co-Author(s)

MF

Marta Fernández

MD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain
TL

Tamara Lado

MD
Servicio de Hematología y Hemoterapia. Complejo Hospitalario Universitario A Coruña
La Coruña, Galicia, Spain
ML

María Fernanda Lopez-Fernandez, MD

PhD/MD
Servicio de Hematologia y Hemoterapia. Complejo Hospitalario Universitario A Coruia
La Coruna, Galicia, Spain

Background: Acquired von Willebrand syndrome (AVWS) is a probably under-diagnosed hemorrhagic condition, clinically similar to congenital von Willebrand disease (VWD), normally associated with other underlying conditions. Diagnostic of AVWS includes clinical symptomatology and a complex battery of laboratory tests. The utility of BAT (ISTH-SCC bleeding assessment tool) questionnaire in the diagnostic of AVWS was not evaluated. In VWD BAT ≥ 3 is considered abnormal.

Aims: Describe path to diagnosis of AVWS and evaluate the utility of BAT questionnaire in AVWS.

Methods: We evaluated BAT questionnaire, and VWF laboratory test in patients with suspicion of AVWS (presence of underlying disease related with AVWS and recent hemorrhagic diathesis or high risk of hemorrhage). Laboratory study included: platelet function assay (PFA), ristocetin induced platelet agglutination (RIPA), VWF antigen (VWF:Ag), VWF ristocetin cofactor (VWF:RCo), VWF collagen binding (VWF:CB) and ratios, and VWF multimeric distribution (SDS-agarose gel). Patients with 2-3 laboratory criteria were included in AVWS group, and with 1-0 criteria, in Control group (Table 1).

Results: A total of 82 patients were enrolled: 68 with CVD (cardiovascular disease), 6 with MG (monoclonal gammopathy), 5 with ET (essential thrombocytemia), 2 with cancer, and 1 with lupus. AVWS was confirmed in 50 (60.9%) patients and Control group 32 (39%). Probability of BAT ≥ 3 being related with AVWS diagnostic was 92% (OR=12.7, CI 95% 3,9 – 40.9). There was significant difference in BAT values comparing AVWS group with Control group (Table 2). AVWS patients that were bleeders in moment of diagnosis had BAT > 3 (mean) in all underlying diseases except for ET. In Control group BAT was ≤ 3 despite presence of bleed.

Conclusion(s): Results suggest that BAT questionnaire is useful for the diagnostic of AVWS. Larger studies are necessary to compare BAT results in different underlying diseases and evaluate utility in hemorrhagic risk evaluation.