Acquired von Willebrand syndrome: Features and management

Mohri, Hiroshi

doi:10.1002/ajh.20455

Cited by 76 publications

(71 citation statements)

References 82 publications

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“…AVWS is caused by a number of mechanisms as reviewed by Mohri 25. There was no clear relationship between VWF Ac/VWF:Ag ratio and multimer profile in our patients however, agreement between multimer methods was observed in 77% of patients.…”

Section: Discussioncontrasting

confidence: 49%

Evaluation of a semi‐automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers

Bowyer¹,

Goodfellow²,

Seidel³

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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BackgroundThe phenotypic diagnosis of von Willebrand disease (VWD) is a multistep process with classification dependent on the quantification of von Willebrand factor (VWF) multimeric structure. VWF multimer analysis is a technically challenging, lengthy and non‐standardised assay, usually performed in specialist laboratories. Recently, a new semi‐automated multimer assay, the Hydragel 5 von Willebrand multimers (H5VWM) has become available.ObjectivesThis study, performed in two European centres, compared existing in‐house multimer assays to the H5VWM in individuals with and without VWD.ResultsOverall agreement of 91.1% was observed in 74 individuals with normal VWF levels, 57 patients grouped as type 1 VWD, 33 type 2A, 16 type 2B, 28 type 2M, 11 type 2N. Patients tested following Desmopressin or VWF concentrate, with thrombotic thrombocytopenic purpura and acquired von Willebrand syndrome were also evaluated. Many of the discrepancies between methods were in patients with genetic mutations linked to more than one type of VWD including p.R1374C/H and p.R1315C. Quantifiable multimer results were available within one working day. Densitometry improved the interpretation of the multimers with slight structural variations that were not apparent by visual inspection of the in‐house method.Conclusions5VWM was a rapid, sensitive, standardised assay which used existing technology and could be included as an initial screen of VWF multimers in a VWD diagnostic algorithm in conjunction with traditional multimer analysis.

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Section: Discussioncontrasting

confidence: 49%

Evaluation of a semi‐automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers

Bowyer¹,

Goodfellow²,

Seidel³

et al. 2018

Research and Practice in Thrombosis and Haemostasis

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“…AVWS results from increased shear stress in VADs and the tubes that connect the VAD to the heart and great vessels. Artificial surfaces and differences in velocity may enhance shear stress, which subsequently leads to the unfolding of Von Willebrand factor (VWF) and cleavage by the protease ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin motif ) [9][10][11] . This process induces the loss of high-molecular weight (HMW) multimers, thus resulting in the decreased binding of VWF to collagen.…”

Section: Introductionmentioning

confidence: 99%

Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy

Kalbhenn

Schmidt

Nakamura

et al. 2015

J Atheroscler Thromb

104

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Aims: Acquired Von Willebrand syndrome (AVWS) is an acquired bleeding disorder that has been reported to aggravate bleeding complications in patients with ventricular assist devices or aortic stenosis. AVWS is characterized by the loss of the high-molecular-weight (HMW) multimers of Von Willebrand factor (VWF) with consequent impaired VWF binding to platelets and collagen. The aim of this study was to investigate the development of AVWS in patients treated with veno-venous ECMO (extracorporeal membrane oxygenation) support. Methods: We examined the presence of AVWS in adult patients receiving ECMO support (n 18) and control subjects treated without ECMO support (n 18). The diagnosis of AVWS was made based on the ratio of collagen-binding capacity to VWF-antigen (VWF:CB/VWF:Ag) and a VWF multimeric analysis. In addition, bleeding episodes were monitored. Results: All patients supported with ECMO developed AVWS. AVWS was identified in the early period after ECMO implantation, i.e. within 24 hours after ECMO implantation. In 17 patients, the VWF:CB/VWF:Ag ratio was significantly reduced and HMW multimers were severely missing, and 17 of the 18 patients developed bleeding complications and required transfusions of blood, FFP and/ or platelet concentrates. In addition, nine patients without an ECMO device were investigated (prior to ECMO implantation: n 2, after ECMO explantation: n 7). Conclusions: In this study, all patients treated with ECMO support developed AVWS, and AVWS was detectable within 24 hours after ECMO implantation. However, the AVWS was reversible after ECMO explantation. Making an early diagnosis of AVWS and providing appropriate treatment may reduce the incidence of life-threatening bleeding. See

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“…The laboratory diagnostic workup of aVWD has not yet been standardized. Reduced von Willebrand factor (VWF) activity, reduced VWF antigen (Ag) levels, a pathologic ratio of VWF activity to VWF:Ag levels and a reduction or absence of high-molecular-weight VWF multimers are often found in patients with aVWD [3,7,8,9]. However, these assays are not useful to differentiate aVWD from congenital forms.…”

Section: Introductionmentioning

confidence: 99%

Causes, Etiology and Diagnosis of Acquired von Willebrand Disease: A Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies

Sucker¹,

Michiels

Zotz³

2009

Acta Haematol

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Acquired von Willebrand disease (aVWD) occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative and myeloproliferative disorders, other malignancies, and cardiovascular disease. aVWD is a complex and heterogeneous defect with a multifactorial etiology and the pathophysiologic mechanisms remain unclear in many cases. Assays for anti-factor VIII (FVIII)/von Willebrand factor (VWF) activities often yield negative results although antibodies may be present in autoimmune disease and some lymphoproliferative disorders. Functional assays of VWF in patients’ plasma and particularly in heart valve disease, VWF multimer analysis are important for aVWD diagnosis. In patients with normal partial thromboplastin times and normal VWF activity, the diagnosis of aVWD is based on clinical suspicion and a careful bleeding history, which should prompt the clinician to initiate further laboratory investigations. Management of bleeding in aVWD relies mainly on desmopressin, FVIII/VWF concentrates and high-dose intravenous immunoglobulin. The half-life of VWF may be very short, and in bleeding episodes high doses of FVIII/VWF concentrates at short intervals may be necessary even when high-dose intravenous immunoglobulin was applied before. Since the optimal treatment strategy has not yet been defined for aVWD of different etiology, controlled multicenter trials aiming at the development of standardized treatment protocols are urgently needed.

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Acquired von Willebrand syndrome: Features and management

Cited by 76 publications

References 82 publications

Evaluation of a semi‐automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers

Evaluation of a semi‐automated von Willebrand factor multimer assay, the Hydragel 5 von Willebrand multimer, by two European Centers

Early Diagnosis of Acquired von Willebrand Syndrome (AVWS) is Elementary for Clinical Practice in Patients Treated with ECMO Therapy

Causes, Etiology and Diagnosis of Acquired von Willebrand Disease: A Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies

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