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acquired von willebrand disease

Hanne Skaadel, Øystein Bruserud
Even though thrombotic events are rare in patients with coagulation deficiencies, several cases of both arterial and venous thromboses have been reported in patients with single coagulation factor defects and platelet defects. Thromboses have been described both in hemophilia A and B, von Willebrand disease as well as in many other rare congenital coagulation factor and platelet defects. Thromboses may also occur in patients with acquired hemophilia and in patients with severe thrombocytopenia due to hematological malignancies or intensive chemotherapy...
January 28, 2019: Expert Review of Hematology
Marie Scully, Spero R Cataland, Flora Peyvandi, Paul Coppo, Paul Knöbl, Johanna A Kremer Hovinga, Ara Metjian, Javier de la Rubia, Katerina Pavenski, Filip Callewaert, Debjit Biswas, Hilde De Winter, Robert K Zeldin
BACKGROUND: In acquired thrombotic thrombocytopenic purpura (TTP), an immune-mediated deficiency of the von Willebrand factor-cleaving protease ADAMTS13 allows unrestrained adhesion of von Willebrand factor multimers to platelets and microthrombosis, which result in thrombocytopenia, hemolytic anemia, and tissue ischemia. Caplacizumab, an anti-von Willebrand factor humanized, bivalent variable-domain-only immunoglobulin fragment, inhibits interaction between von Willebrand factor multimers and platelets...
January 9, 2019: New England Journal of Medicine
Hugo De Larochellière, Rishi Puri, John W Eikelboom, Josep Rodés-Cabau
Transcatheter aortic valve replacement (TAVR) is well established for treating patients with severe aortic stenosis considered at intermediate to high surgical risk. Blood disorders such as anemia, thrombocytopenia, and acquired type 2A von Willebrand disease are relatively frequent in TAVR candidates, and multiple studies to date have highlighted their potential clinical association with mortality and/or bleeding complications post-TAVR. The present review provides an overview of various blood disorders observed pre- and post-TAVR, with special focus on their incidence, etiology, clinical association, and management...
January 14, 2019: JACC. Cardiovascular Interventions
Sarah Bos, Bente van den Boom, Pieter W Kamphuisen, Jelle Adelmeijer, Hans Blokzijl, Tim Schreuder, Ton Lisman
BACKGROUND AND AIM:  Patients with cirrhosis may acquire profound changes in haemostasis. Although haemostatic changes in cirrhosis have been extensively studied, most studies were performed in groups of patients with mixed aetiology. As thrombotic events appear more common in some aetiologies of disease, notably non-alcoholic steatohepatitis (NASH) and cholestatic disease, we hypothesized that haemostatic changes might be different across aetiologies. PATIENTS AND METHODS:  We studied 109 patients with cirrhosis (31 cholestatic liver disease, 23 NASH, 37 alcoholic liver disease [ALD], 18 viral hepatitis) and 44 healthy controls...
January 4, 2019: Thrombosis and Haemostasis
Elizabeth Sarah Mayne, Malcolm Tait, Barry Frank Jacobson, Evashin Pillay, Susan J Louw
Background: Autoimmune paraphenomena, are associated with B-cell lymphoproliferative disorders, including monoclonal gammopathy of uncertain significance. These paraphenomena can rarely include acquired bleeding disorders. Case presentation: This case study reports an unusual clinical presentation of 2 acquired bleeding disorders, Acquired von Willebrand syndrome (disease) and Acquired Glanzmann's thrombasthenia, in an elderly patient with monoclonal gammopathy of uncertain significance...
2018: Thrombosis Journal
O'Brien B, Mason Ja, Kimble Rmn
STUDY OBJECTIVE: Heavy menstrual bleeding (HMB) is a common gynaecological complaint among young females with up to 40% having experienced HMB. Bleeding disorders are increasingly being recognised in adolescents and young adults with HMB. The aim of this study was to determine the prevalence of bleeding disorders in adolescents with HMB, among patients presenting to the Queensland Statewide Paediatric and Adolescent Gynaecology (PAG) Service between July 2007 to July 2017. DESIGN, SETTINGS, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: The study was a retrospective review of 124 adolescent females aged 8 to 18 years with HMB presenting to the Queensland PAG Service, Brisbane, Australia...
November 22, 2018: Journal of Pediatric and Adolescent Gynecology
Mate Petricevic, Jadranka Knezevic, Gordan Samoukovic, Bozena Bradaric, Ivica Safradin, Marija Mestrovic, Vasil Papestiev, Alen Hodalin, Tomislav Madzar, Mario Mihalj, Ante Rotim, Bojan Biocina
The 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...
November 20, 2018: Thoracic and Cardiovascular Surgeon
Makoto Iijima, Nagomi Itoh, Ryota Murase, Yutaka Makino
INTRODUCTION: The combination of aortic stenosis, acquired coagulopathy, and anemia due to gastrointestinal (GI) bleeding is described as Heyde syndrome. PRESENTATION OF CASE: We report a surgical case of a 77-year-old man who was admitted because of melena and exertional chest compression. GI endoscopy could not reveal the origin of the GI bleeding. Conservative therapy including fasting and transfusion improved the anemia. Echocardiography demonstrated severe aortic stenosis (AS) with a hypertrophied left ventricle...
November 10, 2018: International Journal of Surgery Case Reports
Janie Charlebois, Georges-Étienne Rivard, Jean St-Louis
Acquired von Willebrand syndrome (AVWS) is a rare acquired bleeding disorder that resembles von Willebrand disease by its clinical symptoms and laboratory findings, but differs by its negative personal and family history of bleeding diathesis. AVWS is mostly seen in the elderly, but it has been described in children, often in those with congenital heart disease and Wilms tumor. It is most commonly associated with lymphoproliferative, myeloproliferative, cardio-vascular, or autoimmune diseases, solid tumors, and certain drugs...
October 30, 2018: Transfusion and Apheresis Science
Shannen Deconinck, Claudia Tersteeg, Els Bailleul, Leen Delrue, Nele Vandeputte, Inge Pareyn, Nathalie Itzhar-Baikian, Hans Deckmyn, Simon F De Meyer, Marc Vanderheyden, Karen Vanhoorelbeke
Background: Patients with von Willebrand disease (VWD) type 2A or acquired von Willebrand syndrome (aVWS) as a consequence of implantation of left ventricular assist devices (LVAD) are both characterized by a loss of von Willebrand factor (VWF) function. Loss of VWF function is however more severe in VWD type 2A than in LVAD patients. Objectives: To compare VWF function in patients with VWD type 2A and LVAD-induced aVWS to highlight the differences in VWF activity and to stress the importance of VWF multimer analysis for correct diagnosis of aVWS in LVAD patients...
October 2018: Research and Practice in Thrombosis and Haemostasis
Dana Berg, Edward Lebovics, Masashi Kai, David Spielvogel
Heart failure affects over 5 million Americans, with numbers expected to rise. While heart transplantation is the most effective long-term strategy for end-stage heart failure, there is a limited cardiac donor pool, and these organs are often unavailable at the time of need. Left ventricular assist devices (LVADs), therefore, continue to be used to bridge this gap. Originally implanted as a bridge to transplant, these devices are now additionally utilized as destination therapy for patients ineligible for transplant...
October 25, 2018: Cardiology in Review
Annette E Bowyer, Karen J Goodfellow, Holger Seidel, Philipp Westhofen, Francesca Stufano, Anne Goodeve, Stephen Kitchen, Michael Makris
Background: The 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. Objectives: This study, performed in two European centres, compared existing in-house multimer assays to the H5VWM in individuals with and without VWD...
October 2018: Research and Practice in Thrombosis and Haemostasis
Hisanori Horiuchi
The von Willebrand factors (VWFs) play critical role in hemostasis and thrombosis formation. VWFs are produced in and secreted as large multimers from endothelial cells, and shear stress-dependently cleaved into 2-80 multimers by their specific protease, ADATS13. Because high molecular weight VWFs play important roles in platelet aggregation, the loss of high molecular weight VWFs caused by pathological high-shear stress induces a hemostatic disorder known as acquired von Willebrand syndrome (AVWS) type IIA...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Ritsuko Sasaki, Yoshiya Horimoto, Ju Mizuno, Yoko Edahiro, Tsukasa Ohmori, Norio Komatsu, Mitsue Saito
BACKGROUND: Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those of congenital von Willebrand disease (VWD). Patients with aVWS may require prophylactic treatment to prevent excessive bleeding following surgery. To our knowledge, to date, there have been no reports on perioperative management for breast cancer patients with aVWS. CASE PRESENTATION: A 60-year-old woman with breast cancer was diagnosed with aVWS due to polycythemia vera...
September 17, 2018: Surgical Case Reports
John Chapin
Von Willebrand disease (VWD) is an inherited bleeding disorder that affects up to 1% of the population. In most cases, VWD results from a mutation in the von Willebrand Factor (VWF) gene, which alters the amount and function of VWF, a key glycoprotein in both primary and secondary hemostasis. A comprehensive analysis of patients with VWD should include VWF activity, antigen levels, platelet function, and a careful bleeding history. Treatment options include antifibrinolytics, desmopressin, and VWF replacement therapy...
2018: Clinical Interventions in Aging
Omar Alshuwaykh, Michael J Krier
BACKGROUND Heyde syndrome is the association between gastrointestinal (GI) bleeding from arteriovenous malformation (AVM) and aortic stenosis. The aim of this study was to review Heyde syndrome and to discuss the management of this condition. CASE REPORT A 56-year-old female with a history of severe aortic stenosis and recurrent GI bleeding secondary to small bowel AVM, presented for hospital admission with melena and maroon blood in her stool. The patient underwent esophagogastroduodenoscopy with push enteroscopy, full colonoscopy, and mesenteric angiogram with failure to identify any active bleeding sources...
August 7, 2018: American Journal of Case Reports
Brian Odom, Iyad Khourdaji, Victoria Golas, Richard Zekman, Bradley Rosenberg
Background: Acquired von Willebrand disease (AvWD) is a rare and often underdiagnosed disease that typically is associated with lymphoproliferative, cardiovascular disease, and myeloproliferative disease. It is challenging to diagnose as it requires a hemostatic challenge to present itself. Case Presentation: This is a 46-year-old male with a history of multiple sclerosis complicated by neurogenic bladder who presented with intermittent gross painless hematuria. He underwent a gross hematuria workup. Cystoscopy demonstrated active bleeding from the right ureteral orifice...
2018: Journal of Endourology Case Reports
Nadia Aissaoui, Jerome Jouan, Melissa Gourjault, Benoit Diebold, Sofia Ortuno, Amer Hamdan, Christian Latremouille, Romain Pirracchio, Michiel Morshuis
BACKGROUND/AIMS: Long-term mechanical assist devices are now commonly used in the treatment of severe heart failure to unload the failing ventricle, maintain sufficient end-organ perfusion and improve functional capacity. Depending on the assisted ventricles, 3 categories of long-term assist devices are available: left ventricular assist device (LVAD), biventricular assist device and total artificial heart. Improvements in technology, especially the advent of smaller, durable continuous flow pumps, have led to the use of LVADs in a much broader population of patients in the last 10 years...
2018: Blood Purification
Christopher O Austin, Dong Chen, Colleen S Thomas, Robert E Safford, Brian P Shapiro, Justin A Bryan, Jordan C Ray, Joseph L Blackshear
<AbstractText Label="Background" NlmCategory="UNASSIGNED">von Willebrand factor (VWF) multimer quantitation has been utilized in the assessment of valvular heart disease, however, there is no standardized method for quantitation. We compared three methods of assessment which utilized a normal plasma control. Methods: We analyzed 476 samples and their control plasma from 368 patients with valvular heart disease, hypertrophic cardiomyopathy, or LVAD therapy, and 27 normal subjects...
January 2018: Research and Practice in Thrombosis and Haemostasis
Emmanuel J Favaloro, Soma Mohammed, Giuseppe Lippi
von Willebrand disease (VWD) and haemophilia represent the most common inherited or acquired bleeding disorders. However, many laboratories and clinicians may be challenged by their accurate diagnosis or exclusion. Difficulties in diagnosis/exclusion may include analytical issues, where assays occasionally generate an incorrect result (ie representing an analytical error) or have limitations in their measurement range of and/or low analytical sensitivity. Also increasingly recognized is the influence of preanalytical issues on the diagnosis of VWD or haemophilia...
November 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
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