Review
Hematology
Kirstin Sandrock-Lang, Hannah Glonnegger, Barbara Zieger
Summary: Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder caused by various underlying diseases or conditions. The diagnosis of AVWS is challenging and requires comprehensive laboratory investigations and a thorough patient history. Treatment options include tranexamic acid, 1-desamino-8-D-arginine vasopressin, and VWF-containing concentrate.
Article
Hematology
Joppe G. Drop, Enno D. Wildschut, Moniek P. M. de Maat, Joost van Rosmalen, Willem P. de Boode, Matthijs de Hoog, C. Heleen van Ommen
Summary: This study investigated the incidence and longitudinal profile of acquired von Willebrand disease (AVWD) in children receiving ECMO support, as well as its association with bleeding complications. The results showed that all children developed AVWD during ECMO, but AVWD was independent of major bleeding and recovered quickly after ECMO cessation.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Review
Hematology
Ines Ghariani, Nejia Braham, Agnes Veyradier, Leila Bekir
Summary: Acquired von Willebrand syndrome is a rare bleeding disorder that shares similarities with inherited von Willebrand disease. This study presents a retrospective case series of five male patients diagnosed with acquired von Willebrand syndrome between 2010 and 2020. They all exhibited recent mucocutaneous bleeding and had a biological phenotype consistent with type 2 von Willebrand disease. Underlying conditions such as lymphoproliferative, autoimmune, and cardiovascular diseases were identified. Treatment for the underlying disorder, including high-dose intravenous immunoglobulins, resulted in improvement of plasma von Willebrand factor levels in most cases.
THROMBOSIS RESEARCH
(2022)
Article
Hematology
Shannen J. Deconinck, Christoph Nix, Svenja Barth, Eveline Bennek-Schoepping, Antoine Rauch, An-Sofie Schelpe, Elien Roose, Hendrik B. Feys, Inge Pareyn, Aline Vandenbulcke, Joshua Muia, Christophe Vandenbriele, Sophie Susen, Bart Meyns, Claudia Tersteeg, Steven Jacobs, Simon F. De Meyer, Karen Vanhoorelbeke
Summary: Acquired von Willebrand syndrome (aVWS) is common in patients with mechanical circulatory support devices and can lead to bleeding complications. Our study demonstrates that inhibiting ADAMTS13 with an anti-ADAMTS13 monoclonal antibody can prevent the degradation of von Willebrand factor (VWF) and improve hemostasis in these patients.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Snjezana Janjetovic, Christina Charlotte Rolling, Ulrich Budde, Sonja Schneppenhem, Philippe Schafhausen, Mia Carlotta Peters, Carsten Bokemeyer, Katharina Holstein, Florian Langer
Summary: This study compared different diagnostic tools for detecting MPN-associated aVWS. Results showed that using multimer analysis of plasma VWF, aVWS with a loss of VWF high-molecular-weight multimers was detected in 51.4% and 55.6% of PV and ET patients, respectively. A VWF:GPIbM/Ag threshold of <0.8 was identified as a useful screening tool for aVWS, with good sensitivity (88.2%) and specificity (76.7%) and agreement with VWF multimer analysis (Kappa = 0.653). VWF:GPIbM/Ag outperformed VWF:CB/Ag. Hematocrit did not differ between patients with and without aVWS, but leukocyte and platelet counts were significantly increased in the aVWS cohort.
THROMBOSIS RESEARCH
(2022)
Article
Hematology
Pierre Laporte, Marie Tuffigo, Anne Ryman, Mathieu Fiore, Etienne Riviere, Chloe James, Alexandre Guy
Summary: The study aimed to evaluate the performance of VWF:GPIbR with HemosIL VWF:RCo Werfen versus VWF:RCo in patients with thrombocytosis in the context of MPNs and in patients with secondary thrombocytosis.
THROMBOSIS AND HAEMOSTASIS
(2022)
Review
Cardiac & Cardiovascular Systems
Haiwang Wang, Duo Li, Yuansen Chen, Ziquan Liu, Yanqing Liu, Xiangyan Meng, Haojun Fan, Shike Hou
Summary: Extracorporeal membrane oxygenation (ECMO) is a life-saving mechanical assistance system that provides support for reversible or treatable diseases. However, bleeding remains a common complication associated with increased morbidity and mortality. Acquired von Willebrand syndrome (AVWS), caused by shear-induced deficiency of von Willebrand factor (VWF), is suggested as one of the causes of bleeding. The mechanisms of VWF deficiency under ECMO support and the association between AVWS and bleeding complications are still unknown. This review discusses VWF loss caused by shear induction in the context of ECMO support and current diagnostic and management strategies for AVWS.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Article
Hematology
Maria Poza, Rodrigo Iniguez, Irene Zamanillo, Sara Redondo, Rafael Alonso, Joaquin Martinez-Lopez, Ana Jimenez-Ubieto
Summary: In Waldenstrom macroglobulinemia patients with acquired von Willebrand syndrome, treatment with ibrutinib may lead to a rapid increase in von Willebrand factor levels, improving bleeding symptoms despite only achieving partial response in IgM levels. This improvement could be attributed to reduced glycoprotein Ib receptor expression induced by ibrutinib and subsequent increase in von Willebrand factor levels in the blood.
THERAPEUTIC ADVANCES IN HEMATOLOGY
(2021)
Article
Hematology
Masayuki Kubo, Kazuya Sakai, Masaki Hayakawa, Hirokazu Kashiwagi, Hideo Yagi, Yoshinobu Seki, Atsushi Hasegawa, Haruyuki Tanaka, Itsuto Amano, Yoshiaki Tomiyama, Masanori Matsumoto
Summary: This study investigated whether VWF degradation is enhanced in patients with ET. The results showed that patients with higher platelet counts had lower HMW-VWFM index and higher VWF-DP:VWF Ag ratio. Cytoreductive therapy increased the HMW-VWFM index and decreased the VWF-DP:VWF Ag ratio. In conclusion, an increased platelet count in ET patients is associated with enhanced cleavage of VWF, and cytoreductive therapy can reduce this cleavage and improve VWFM distributions.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Michael Iarossi, Cedric Hermans
Summary: Acquired von Willebrand syndrome is a rare condition with various etiologies, including lymphoproliferative and myeloproliferative syndromes, as well as cardiac diseases. Viral infections, although extremely rare, can also be a cause, as seen in a reported case after EBV infection. This case report suggests a likely association between SARS-CoV2 infection and the development of a time-limited acquired von Willebrand syndrome.
ACTA HAEMATOLOGICA
(2023)
Review
Medical Laboratory Technology
Ines Ghariani, Nejia Jmili Braham, Leila Bekir
Summary: Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder that often manifests in late adulthood. The disease is associated with increased degradation or clearance of circulating vWF. Diagnosis and treatment are challenging due to the need for multiple laboratory tests and the typically serious underlying conditions of the elderly population affected.
ANNALES DE BIOLOGIE CLINIQUE
(2022)
Article
Anesthesiology
Michael Mazzeffi, Allison Bathula, Ali Tabatabai, Jay Menaker, David Kaczorowski, Ronson Madathil, Samuel Galvagno, Chetan Pasrija, Raymond Rector, Kenichi Tanaka, Daniel Herr
Summary: This study examined the use of VWF concentrate in adult ECMO patients with acquired VWS-related bleeding, finding that it can improve laboratory parameters and shorten the resolution time for bleeding in patients.
JOURNAL OF CARDIOTHORACIC AND VASCULAR ANESTHESIA
(2021)
Article
Medicine, General & Internal
Katharina Meinel, Felicitas Korak, Martin Dusleag, Tanja Strini, Daniela Baumgartner, Ante Burmas, Hannes Sallmon, Barbara Zieger, Axel Schlagenhauf, Martin Koestenberger
Summary: This study explores the relationship between hemodynamic alterations in Fontan patients and hemostatic dysbalance and Fontan-associated liver disease. The accumulation of bile acids is found to be related to certain hemostatic factors, and aspirin may reduce complications. Screening for AVWS is recommended for Fontan patients.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Pediatrics
Ivonne Wieland, Franziska Diekmann, Julia Carlens, Laura Hinze, Katharina Lambeck, Thomas Jack, Georg Hansmann
Summary: This study aims to investigate the link between acquired von Willebrand Syndrome (AVWS) type 2 and pulmonary hypertension (PH). By retrospectively evaluating the charts of 20 children with PH, the study found that 82% of the children had AVWS, with most of them being AVWS type 2. The study suggests systematic analysis of von Willebrand variables in children with PH to prevent bleeding complications after surgery or trauma.
FRONTIERS IN PEDIATRICS
(2022)
Article
Medicine, General & Internal
Manuela Andrea Hoffmann, Sarah N. Knoll, Pia-Elisabeth Baque, Florian Rosar, Inge Scharrer, Stefan Reuss, Mathias Schreckenberger
Summary: This prospective study aimed to investigate the impact of hypothyroid metabolic status on the coagulation and fibrinolytic system, and its association with acquired von Willebrand syndrome (VWS-ac). The findings suggest that hypothyroid patients may have impaired coagulation function and an increased risk of VWS-ac.
JOURNAL OF CLINICAL MEDICINE
(2023)