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Published on: August 14, 2017
Acquired von Willebrand syndrome in multiple myeloma
Irena Djunic1, Ivo Elezovic, Vesna Ilic
1Clinic for Haematology, Clinical Center of Serbia, Belgrade, Serbia. irenadju@eunet.rs
Abstract:
Acquired von Willebrand syndrome (AvWS) is an uncommon complication of multiple myeloma (MM), and it is believed to be connected with paraprotein. The aim of this study was to determine the incidence of AvWS in patients with MM, and estimate the role of paraprotein in its occurrence. The study included 40 patients with MM. The plasma level of paraprotein, platelet adhesion on glass pearls, plasma von Willebrand factor antigen concentration, and ristocetin-induced platelet aggregation (RIPA) were measured initially. Absence of RIPA was found in six patients with MM (15%); however, all six of them had normal levels of von Willebrand factor antigen. Paraprotein was isolated from the serum of these patients. Platelet aggregation was measured in six healthy donors before and after addition of the isolated paraprotein. RIPA was significantly decreased in healthy donors in the presence of paraprotein (P<0·001). The same test was repeated with added human immunoglobulins for intravenous use without any change in RIPA. A significant negative correlation between plasma paraprotein level and RIPA was found (P<0·001). These investigations have shown that paraprotein is associated with AvWS in patients with MM.
Insights
Acquired von Willebrand syndrome (AvWS) in multiple myeloma (MM) patients is linked to paraprotein. This study found paraprotein significantly impairs platelet aggregation, confirming its role in AvWS development.
Area of Science:
- Hematology
- Oncology
Background:
- Acquired von Willebrand syndrome (AvWS) is a rare complication associated with multiple myeloma (MM).
- The underlying mechanism linking AvWS to MM, particularly the role of paraprotein, requires further elucidation.
Purpose of the Study:
- To determine the incidence of AvWS in patients diagnosed with multiple myeloma.
- To investigate the specific role of paraprotein in the pathogenesis of AvWS in MM patients.
Main Methods:
- Assessed 40 MM patients for AvWS markers including paraprotein levels, von Willebrand factor antigen, platelet adhesion, and ristocetin-induced platelet aggregation (RIPA).
- Isolated paraprotein from patient serum and tested its effect on platelet aggregation in healthy donors.
- Compared RIPA in donors with and without added paraprotein, and with added intravenous immunoglobulins.
Main Results:
- Six out of 40 MM patients (15%) exhibited absent RIPA, despite normal von Willebrand factor antigen levels.
- Addition of isolated paraprotein to healthy donor plasma significantly reduced RIPA (P<0.001).
- A significant negative correlation was observed between plasma paraprotein levels and RIPA (P<0.001).
Conclusions:
- Paraprotein is directly associated with the development of acquired von Willebrand syndrome in multiple myeloma.
- The presence of paraprotein impairs platelet aggregation, explaining its role in AvWS pathophysiology.
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