VPB0003 - Acquired von Willebrand syndrome in patients with monoclonal gammopathy: a single centre experience.

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) related with lymphoproliferative disorders (LD) is more frequent in patients with monoclonal gammopathy of undetermined significance (MGUS). The pathophysiological mechanisms can be autoantibodies against von Willebrand factor (VWF), or inmuno-absorption by plasmatic cells. Diagnostic is complex and bleeding can be severe. The answer to different treatments is not fully established.

Aims: Describe the path to diagnosis of AVWS in patients with monoclonal gammopathy (MG) and the therapeutic approaches.

Methods: We have enrolled five patients with monoclonal component IgG or IgA without previous or family history of bleeding, who presented recent significant hemorrhage.We assessed BAT (ISTH-SCC bleeding assessment tool) questionnaire and evaluated the hemorrhagic diathesis. Laboratory tests included primary and secondary hemostasis tests, and complete VWF study including multimeric analysis of VWF, and inhibitor to VWF. We evaluated the response to hemostatic treatments, and treatment of underlying disease.

Results: BAT results were > 3 (normal value < 3) in 4 of 5 patients (median 5.2); 3 patients needed hospitalization, blood products and FVIII/FVW concentrates administration, due to hemorrhagic episodes. VWF levels were very low. All patients had loss of VWF multimers. VWF inhibitor tests were negative. The response to IVIg (intravenous inmunoglobulins) was variable and transitory within 2 to 3 weeks. The answer to desmopressin and FVIII/VWF concentrates was poor or partial, due to the accelerated clearance of VWF. Response to the treatment of underlying disease was null, including: bortezomib, dexamethasone and cyclophosphamide; lenalidomide, and allogeneic bone marrow transplant.

Conclusion(s): Results suggest accelerated clearance of VWF multimers probably by absorption by malignant cells. Bleeding is frequent, can be severe, and sometimes blood products are needed. IVIg is the best option, but transitory. Response to treatment of underlying disease is null.