Acquired von Willebrand Syndrome in Pediatric Patients during Mechanical Circulatory Support

Kubicki, Rouven; Stiller, Brigitte; Kroll, Johannes; Siepe, Matthias; Beyersdorf, Friedhelm; Benk, Christoph; Hohn, R B; Grohmann, Jochen; Fleck, Thilo; Zieger, Barbara

doi:10.1055/s-0039-1678862

Cited by 4 publications

(5 citation statements)

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“…Acquired von Willebrand syndrome occurs during ECMO due to a loss of high molecular weight VWF multimers from shear stress which disrupts VWF multimers and is associated with increased bleeding complications. 48,49 Shear stress also causes platelets to produce small circulating fragments of platelet plasma membranes called platelet microparticles which contribute to thrombus formation. 50 Platelet microparticles are increased in neonatal ECMO systems in vivo but no study to date has demonstrated that they contribute to a prothrombotic state in vitro.…”

Section: Platelet Functionmentioning

confidence: 99%

Platelet Count and Function during Pediatric Extracorporeal Membrane Oxygenation

Cashen

Meert

Dalton

2020

Semin Thromb Hemost

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Extracorporeal membrane oxygenation (ECMO) is a form of life support used to treat neonates, children, and adults with cardiorespiratory failure refractory to conventional therapy. This therapy requires the use of anticoagulation to prevent clotting in the extracorporeal circuit, but anticoagulation also increases the risk of bleeding on ECMO. Both bleeding and thrombosis remain significant complications on ECMO and balancing these risks is challenging. Acquired platelet dysfunction is common during ECMO and quantitative and qualitative platelet dysfunction contributes to bleeding risk. Optimal platelet count, function, and transfusion thresholds are not well established during pediatric ECMO. In this review, we provide an overview of hemostatic alterations during ECMO, changes in platelet count and function, platelet monitoring techniques, bleeding risk, and future needs to best optimize patient management and care.

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Section: Platelet Functionmentioning

confidence: 99%

Platelet Count and Function during Pediatric Extracorporeal Membrane Oxygenation

Cashen

Meert

Dalton

2020

Semin Thromb Hemost

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“…1 42 45 49 51 154 However, much more recent focus on AVWS has recently arisen as a result of the increasing use of MCS, such as VAD and ECMO. 156 188 189 207 239 260 273 281 290 292 294 298 320 AVWS can arise due to absorption of VWF, preferentially HMWM, onto artificial surfaces, or due to increased shear stress. The VWF:CB has been increasingly investigated in this arena, and shown to correlate with loss of HMWM, development of AVWS, and also increased bleeding risk.…”

Section: Discussionmentioning

confidence: 99%

“…Yan et al 289 reported the establishment of a flow cytometric immunobead assay to detecting plasma VWF activity (VWF:GPIbR-like assay) and showed its clinical application in the prognosis of ischemic stroke, as compared with VWF:Ag, VWF:GPIbR, and VWF:CB. Kubicki et al 290 included the VWF:CB in their investigation into AVWS in 39 pediatric patients during mechanical circulatory support (MCS) (extracorporeal life support, n = 13; ECMO, n = 5; and VAD, n = 12). All children developed AVWS during MCS, usually during the early postoperative course, but no AVWS after device explantation.…”

Section: A History Of the Von Willebrand Factor Collagen-binding Assaymentioning

confidence: 99%

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

Favaloro

2023

Semin Thromb Hemost

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The von Willebrand factor (VWF) collagen binding (VWF:CB) assay was first reported for use in von Willebrand diagnostics in 1986, by Brown and Bosak. Since then, the VWF:CB has continued to be used to help diagnose von Willebrand disease (VWD) (correctly) and also to help assign the correct subtype, as well as to assist in the monitoring of VWD therapy, especially desmopressin (DDAVP). However, it is important to recognize that the specific value of any VWF:CB is predicated on the use of an optimized VWF:CB, and that not all VWF:CB assays are so optimized. There are some good commercial assays available, but there are also some “not-so-good” commercial assays available, and these may continue to give the VWF:CB “a bad reputation.” In addition to VWD diagnosis and management, the VWF:CB found purpose in a variety of other applications, from assessing ADAMTS13 activity, to investigation into acquired von Willebrand syndrome (especially as associated with use of mechanical circulatory support or cardiac assist devices), to assessment of VWF activity in disease states in where an excess of high-molecular-weight VWF may accumulate, and lead to increased (micro)thrombosis risk (e.g., coronavirus disease 2019, thrombotic thrombocytopenic purpura). The VWF:CB turns 37 in 2023. This review is a celebration of the utility of the VWF:CB over this nearly 40-year history.

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“…Therefore and due to the more feasible problem of a low factor VIII level no more analysis of von Willebrand factor multimers was performed. 13,14…”

Section: Discussionmentioning

confidence: 99%

ABO incompatibility and mild factor VIII deficiency in the neonate: Not too much of a problem but a challenge on ECMO

et al. 2021

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Coagulopathy and bleeding on extracorporeal membrane oxygenation (ECMO) contribute to a worse outcome, and hyperbilirubinemia is an additional threat for newborn babies. We report a case of a newborn boy with congenital diaphragmatic hernia (CDH) associated with ABO incompatibility and an inherited mild hemophilia A. Due to respiratory failure he needed ECMO on his first day of life. During ECMO an exchange transfusion was performed after an extensive hyperbilirubinemia had evolved. Thereafter severe bleeding occurred, and a very low factor VIII level was found causative for that. After factor VIII substitution bleeding was under control and the baby eventually could be weaned from ECMO, underwent corrective surgery, and recovered.

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Acquired von Willebrand Syndrome in Pediatric Patients during Mechanical Circulatory Support

Cited by 4 publications

References 0 publications

Platelet Count and Function during Pediatric Extracorporeal Membrane Oxygenation

Platelet Count and Function during Pediatric Extracorporeal Membrane Oxygenation

The Role of the von Willebrand Factor Collagen-Binding Assay (VWF:CB) in the Diagnosis and Treatment of von Willebrand Disease (VWD) and Way Beyond: A Comprehensive 36-Year History

ABO incompatibility and mild factor VIII deficiency in the neonate: Not too much of a problem but a challenge on ECMO

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