scholarly journals A Case of Acquired von Willebrand Disease Secondary to Myeloproliferative Neoplasm

2020 ◽  
Vol 13 (2) ◽  
pp. 733-737 ◽  
Author(s):  
Sreethish Sasi ◽  
Mohamed A. Yassin ◽  
Afraa M. Fadul
Keyword(s):  
Early Recognition ◽  
Myeloproliferative Neoplasm ◽  
Myeloproliferative Disorders ◽  
Acute Onset ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
History Of ◽  
Underlying Disorder ◽  
Von Willebrand ◽  
Willebrand Factor

Acquired von Willebrand Disease (AVWD) is a rare disorder in which qualitative or quantitative defects in von Willebrand factor (VWF) occur secondary to other conditions. AVWD occurs in patients with myeloproliferative disorders due to formation of autoantibodies against VWF and development of excessive shear stress causing disruption of VWF multimers. AVWD is different from congenital VWD in its acute onset and absence of family history. We report a 42-year-old gentleman with essential thrombocythemia, who was on cytoreductive therapy with hydroxyurea, and presented with an acute history of gum bleeding with hemoptysis, without any antecedent trauma or infections. His platelet count was very high, and prothrombin time and activated partial thromboplastin time were prolonged. The VWF ristocetin cofactor assay (VWF: RCo) was low, but VWF antigen level (VWF: Ag) was normal. Their ratio (VWF: RCo/VWF: Ag) was much lower than the acceptable lower limit. Treatment in AVWD is focused on addressing the underlying disorder. Early recognition of AVWD and its primary cause is mandatory in providing adequate therapy and achieving a cure.

scholarly journals Acquired von Willebrand disease: from theory to practice. A single center experience - three case reports

2016 ◽  
Vol 24 (1) ◽  
pp. 93-102
Author(s):  
Andrei Colită ◽  
Carmen Saguna ◽  
Andra Costache ◽  
Gabriela Borsaru ◽  
Raluca Manolache ◽  
...  
Keyword(s):  
Case Reports ◽  
Von Willebrand Disease ◽  
Hospital Department ◽  
Single Center Experience ◽  
Acquired Von Willebrand Disease ◽  
History Of ◽  
Underlying Diseases ◽  
Bleeding History ◽  
Von Willebrand ◽  
Willebrand Factor

Abstract Acquired von Willebrand disease (AvWD) represents a rare, potentially severe and most likely underdiagnosed category of hemorrhagic syndromes determined by quantitative, qualitative or functional, nonhereditary, alterations of von Willebrand factor (vWF) that occur in the context of various underlying diseases. It is diagnosed mainly in adults, without any personal or familial history of bleeding. The etiopathogeny of AvWD is complex, marked by the intervention of multiple mechanisms, occuring in the evolution of neoplasia, autoimmune disorders, cardiovascular diseases and other conditions. The clinical and laboratory manifestations are similar to the congenital form with mucocutaneous hemorrhage in patients without bleeding history and demonstration of quantitative and/or functional anomalies of vWF. Treatment has two major objectives: control of bleeding and therapy of the underlying condition. As a practical illustration of the theoretical aspects we present 3 clinical cases of AvWD diagnosed in the Colţea Hospital Department of Hematology during the last 10 years.

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.

scholarly journals Successful management of multiple pregnancies in a family with varying severity of Von Willebrand disease

2018 ◽  
Vol 11 (4) ◽  
pp. 192-194
Author(s):  
Patrick Harrington ◽  
Pippa Kyle ◽  
Jacky Cutler ◽  
Bella Madan
Keyword(s):  
Von Willebrand Factor ◽  
Von Willebrand Disease ◽  
The Other ◽  
Severe Phenotype ◽  
Mild Phenotype ◽  
History Of ◽  
Specialist Treatment ◽  
Factor Concentrate ◽  
Von Willebrand ◽  
Willebrand Factor

We present the obstetric history of a family of three sisters with Von Willebrand disease, managed in our centre over the course of nine successful pregnancies. The abnormalities result from inheritance of an exon 50 skipping mutation in the Von Willebrand factor gene, resulting from consanguinity. Two of the sisters were identified as having a severe phenotype with a Von Willebrand factor level of less than 5 IU/dl, with the other having a mild phenotype. Of the sisters with a severe phenotype, one had a number of prenatal complications and required early onset prophylaxis with Von Willebrand factor concentrate, whilst the other had a less complicated clinical course, only requiring Von Willebrand factor concentrate to cover labour. The sister with mild Von Willebrand disease had a rise in Von Willebrand factor levels during pregnancy and required no specialist treatment. The report highlights the markedly different clinical courses that can occur in patients with Von Willebrand disease and the different approaches to management.

Spontaneous pyohaemothorax in a teenager with von Willebrand disease: a case report and review of literature

2021 ◽  
Vol 14 (8) ◽  
pp. e241613
Author(s):  
Vaishnavi Divya Nagarajan ◽  
Asha Shenoi ◽  
Lucy Burgess ◽  
Vlad C Radulescu
Keyword(s):  
Case Report ◽  
Von Willebrand Factor ◽  
Von Willebrand Disease ◽  
Surgical Drainage ◽  
Review Of Literature ◽  
History Of ◽  
Factor Concentrate ◽  
Type 3 ◽  
Von Willebrand ◽  
Willebrand Factor

An 18-year-old man with a history of type 3 von Willebrand disease (VWD) presented with a spontaneous pyohaemothorax. Type 3 VWD may present with both mucocutaneous and deep-seated bleeds, such as visceral haemorrhages, intracranial bleeds and haemarthrosis. There have been very few cases described in children of spontaneous pyohaemothorax. Management of this patient was challenging due to risks of bleeding following surgical drainage, requiring constant replacement with von Willebrand factor concentrate, while monitoring factor VIII levels to balance the risks of thrombosis.

Acquired von Willebrand Disease in Myeloproliferative Disorders

1996 ◽  
Vol 22 (sup1) ◽  
pp. 79-82 ◽  
Author(s):  
Perry J. J. Van Genderen ◽  
Henriette Leenknegt ◽  
Jan J. Michiels ◽  
Ulrich Budde
Keyword(s):  
Myeloproliferative Disorders ◽  
Von Willebrand Disease ◽  
Acquired Von Willebrand Disease ◽  
Von Willebrand

Abstract 13941: The Prevalence of Cardiovascular Disease in Patients With Von Willebrand Disease; A Potential Therapeutic Target

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Nawfal Mihyawi ◽  
MUHAMMAD AJMAL ◽  
Jun Hung ◽  
Beeletsega T Yeneneh
Keyword(s):  
Cardiovascular Disease ◽  
Therapeutic Target ◽  
Multivariate Logistic Regression Analysis ◽  
Von Willebrand Disease ◽  
Cvd Risk ◽  
International Classification Of Disease ◽  
History Of ◽  
Von Willebrand ◽  
Willebrand Factor

Introduction: Von Willebrand factor (vWF) plays an important role in the coagulation process at the site of vascular injury. It is unclear whether vWF deficiency is protective against cardiovascular disease (CVD) since investigations are limited in this entity. Hypothesis: Our study assessed the hypothesis that the prevalence of CVD is less in patients with than without Von Willebrand disease (vWD). Methods: We queried the National Inpatient Sample (NIS) from 2009-2014. Entire data was used with exclusion of patients below 18 and over 75-year-old. We defined cardiovascular disease as any event of myocardial infarction or a diagnosis of ischemic heart disease using the International Classification of Disease, 9th edition (ICD-9). The primary outcome of interest was to compare the odds ratio of CVD in patients with and without vWD. Results: A total of 224,475,443 weighted hospital discharge samples were identified. Of these, 82,809 carried a diagnosis of vWD. The odds of CVD were less common in vWD patients (OR 0.54; 95% CI 0.52 - 0.56) (table 1). After multivariate logistic regression analysis, with adjustment for age, gender and the most common CVD risk factors including hypertension, smoking, diabetes, hyperlipidemia, family history of CVD, chronic kidney disease and obesity the likelihood of CVD remained lower in vWD patients (OR 0.65; 95% CI 0.63-0.67) (table 2). Conclusions: Our study, the first to involve a large cohort of patients, demonstrated that vWF deficiency is associated with decreased prevalence of CVD. Hence, vWF could potentially be a therapeutic target in CVD management. More investigations are needed for confirmation of these findings and to determine causation.

A novel ELISA-based diagnosis of acquired von Willebrand disease with increased VWF proteolysis

2016 ◽  
Vol 115 (05) ◽  
pp. 950-959 ◽  
Author(s):  
Antoine Rauch ◽  
Claudine Caron ◽  
Flavien Vincent ◽  
Emmanuelle Jeanpierre ◽  
Catherine Ternisien ◽  
...  
Keyword(s):  
Left Ventricular ◽  
Von Willebrand Disease ◽  
Ventricular Assist ◽  
Acquired Von Willebrand Syndrome ◽  
Acquired Von Willebrand Disease ◽  
Medical Need ◽  
Reliable Assessment ◽  
Left Ventricular Assist ◽  
Von Willebrand ◽  
Willebrand Factor

SummaryVon Willebrand disease-type 2A (VWD-2A) and acquired von Willebrand syndrome (AVWS) due to aortic stenosis (AS) or left ventricular assist device (LVAD) are associated with an increased proteolysis of von Willebrand factor (VWF). Analysis of VWF multimeric profile is the most sensitive way to assess such increased VWF-proteolysis. However, several technical aspects hamper a large diffusion among routine diagnosis laboratories. This makes early diagnosis and early appropriate care of increased proteolysis challenging. In this context of unmet medical need, we developed a new ELISA aiming a quick, easy and reliable assessment of VWF-proteolysis. This ELISA was assessed successively in a LVAD-model, healthy subjects (n=39), acquired TTP-patients (n=4), VWD-patients (including VWD-2A(IIA), n=22; VWD-2B, n=26; VWD-2A(IIE), n=21; and VWD-1C, n=8) and in AVWS-patients (AS, n=9; LVAD, n=9; and MGUS, n=8). A standard of VWF-proteolysis was specifically developed. Extent of VWF-proteolysis was expressed as relative percentage and as VWF proteolysis/VWF:Ag ratio. A speeddependent increase in VWF-proteolysis was assessed in the LVAD model whereas no proteolysis was observed in TTP-patients. In VWDpatients, VWF-proteolysis was significantly increased in VWD-2A(IIA) and VWD-2B and significantly decreased in VWD-2A(IIE) versus controls (p< 0.0001). In AVWS-patients, VWF-proteolysis was significantly increased in AS- and LVAD-patients compared to controls (p< 0.0001) and not detectable in MGUS-patients. A significant increase in VWFproteolysis was detected as soon as three hours after LVAD implantation (p< 0.01). In conclusion, we describe a new ELISA allowing a rapid and accurate diagnosis of VWF-proteolysis validated in three different clinical situations. This assay represents a helpful alternative to electrophoresis-based assay in the diagnosis and management of AVWS with increased VWF-proteolysis.Supplementary Material to this article is available online at www.thrombosis-online.com.

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