scholarly journals Reduced Activity of von Willebrand Factor after Flow-Diverting Stent Implantation for Intracranial Aneurysms: A Link to Acquired von Willebrand Disease?

2020 ◽  
Vol 41 (1) ◽  
pp. 140-146 ◽  
Author(s):  
I. Oran ◽  
C. Cinar ◽  
H. Bozkaya ◽  
M. Parildar ◽  
S. Duman
Keyword(s):  
Von Willebrand Factor ◽  
Stent Implantation ◽  
Intracranial Aneurysms ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand ◽  
Willebrand Factor ◽  
Reduced Activity ◽  
Flow Diverting Stent

scholarly journals Acquired von Willebrand disease caused by an autoantibody selectively inhibiting the binding of von Willebrand factor to collagen

Blood ◽  
1994 ◽  
Vol 84 (10) ◽  
pp. 3378-3384 ◽  
Author(s):  
PJ van Genderen ◽  
T Vink ◽  
JJ Michiels ◽  
MB van 't Veer ◽  
JJ Sixma ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Binding Activity ◽  
Von Willebrand Disease ◽  
Low Grade ◽  
Bleeding Tendency ◽  
Glycoprotein Ib ◽  
Acquired Von Willebrand Disease ◽  
Recurrent Epistaxis ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract An 82-year-old man with a low-grade malignant non-Hodgkin lymphoma and an IgG3 lambda monoclonal gammopathy presented a recently acquired bleeding tendency, characterized by recurrent epistaxis, easy bruising, and episodes of melena, requiring packed red blood cell transfusions. Coagulation studies showed a von Willebrand factor (vWF) defect (Ivy bleeding time, > 15 minutes; vWF antigen [vWF:Ag], 0.08 U/mL; ristocetin cofactor activity [vWF:RCoF], < 0.05 U/mL; collagen binding activity [vWF:CBA], 0.01 U/mL; absence of the high molecular weight multimers of vWF on multimeric analysis). Mixing experiments suggested the presence of an inhibitor directed against the vWF:CBA activity of vWF without significantly inhibiting the FVIII:C, vWF:Ag, and vWF:RCoF activities. The inhibitor was identified as an antibody of the IgM class by immunoabsorption of vWF and inhibitor-vWF complexes from the plasma of the patient. Subsequent immunoprecipitation experiments using recombinant fragments of vWF showed that the inhibitor reacted with both the glycoprotein Ib binding domain (amino acids [aa] 422–826) and the A3 (aa 909–1112) domain of vWF, but not with the A2 (aa 716–908) or D4 (aa 1183–1535) domains. We conclude that the IgM autoantibody inhibits the vWF:CBA activity by reacting with an epitope present on both the glycoprotein Ib and A3 domains of vWF.

Diagnosis and Management of Acquired von Willebrand Disease in Heart Disease: A Review of the Literature

2018 ◽  
Vol 68 (03) ◽  
pp. 200-211
Author(s):  
Mate Petricevic ◽  
Jadranka Knezevic ◽  
Gordan Samoukovic ◽  
Bozena Bradaric ◽  
Ivica Safradin ◽  
...  
Keyword(s):  
Heart Disease ◽  
Von Willebrand Factor ◽  
Structural Heart Disease ◽  
Von Willebrand Disease ◽  
Circulatory Support ◽  
Mechanical Destruction ◽  
Acquired Von Willebrand Syndrome ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand ◽  
Willebrand Factor

AbstractThe incidence of acquired von Willebrand syndrome (AvWS) in patients with heart disease is commonly perceived as rare. However, its occurrence is underestimated and underdiagnosed, potentially leading to inadequate treatment resulting in increased morbidity and mortality.In patients with cardiac disease, AvWS frequently occurs in patients with structural heart disease and in those undergoing mechanical circulatory support (MCS).The clinical manifestation of an AvWS is usually characterized by apparent or occult gastrointestinal (GI) or mucocutaneous hemorrhage frequently accompanied by signs of anemia and/or increased bleeding during surgical procedures. The primary change is loss of high-molecular weight von Willebrand factor multimers (HMWM). Whereas the loss of HMWM in patients with structural heart disease is caused by increased HMWM cleavage by von Willebrand factor (vWF)-cleaving protease, ADAMTS13, AvWS in MCS patients is predominantly a result of a high shear stress coupled with mechanical destruction of vWF itself.This manuscript provides a comprehensive review of the evidence regarding both diagnosis and contemporary management of AVWS in patients with heart disease.

Acquired von willebrand disease in multiple myeloma secondary to absorption of von willebrand factor by plasma cells

1990 ◽  
Vol 35 (2) ◽  
pp. 114-117 ◽  
Author(s):  
C. Richard ◽  
M. A. Cuadrado ◽  
M. Prieto ◽  
J. Batlle ◽  
M. Flópez Fernández ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Von Willebrand Factor ◽  
Plasma Cells ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand ◽  
Willebrand Factor

High-dose Intravenous Immunoglobulin Delays Clearance of von Willebrand Factor in Acquired von Willebrand Disease

1995 ◽  
Vol 73 (05) ◽  
pp. 891-892 ◽  
Author(s):  
Perry J J van Genderen ◽  
Wim Terpstra ◽  
Jan J Michiels ◽  
Lian Kapteijn ◽  
Huub H D M van Vliet
Keyword(s):  
Intravenous Immunoglobulin ◽  
Von Willebrand Factor ◽  
Von Willebrand Disease ◽  
High Dose ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand ◽  
Willebrand Factor

scholarly journals Continuous-infusion von Willebrand factor concentrate is effective for the management of acquired von Willebrand disease

2021 ◽  
Vol 5 (14) ◽  
pp. 2813-2816
Author(s):  
Kathryn E. Dane ◽  
John P. Lindsley ◽  
Thomas Kickler ◽  
Michael B. Streiff ◽  
Alison Moliterno ◽  
...  
Keyword(s):  
Continuous Infusion ◽  
Von Willebrand Factor ◽  
Bleeding Risk ◽  
Von Willebrand Disease ◽  
Inadequate Response ◽  
Intermittent Bolus ◽  
Acquired Von Willebrand Disease ◽  
Factor Concentrate ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract Acquired von Willebrand disease (aVWD) is a rare disorder associated with a reduction in von Willebrand factor (VWF) activity, leading to increased bleeding risk. Monoclonal gammopathy of undetermined significance (MGUS) is the most common cause of lymphoproliferative disorder-associated aVWD and is caused by accelerated clearance of circulating VWF. Standard VWF replacement protocols for congenital VWD based on intermittent bolus dosing are typically less effective for aVWD because of antibody-mediated clearance. Intermittent bolus dosing of VWF concentrates often leads to inadequate peak response and profoundly shortened VWF half-life in aVWD. Intravenous immune globulin (IVIG) has demonstrated efficacy in aVWD; however, treatment effect is delayed up to 4 days, limiting its efficacy in acutely bleeding patients. We report the successful use of continuous-infusion VWF concentrate (with or without concomitant IVIG) in 3 patients with MGUS-associated aVWD who had demonstrated an inadequate response to bolus dosing. VWF concentrate doses required in this cohort were higher than typical doses for bleeding treatment in congenital VWD. This report illustrates that continuous-infusion VWF concentrate administration with or without intravenous immunoglobulin rapidly achieves target ristocetin cofactor activity and provides adequate hemostasis in aVWD associated with immunoglobulin G MGUS.

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