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acquired von willebrand disease

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...
July 19, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Ichiro Kawashima, Katsuhiro Takano, Takuma Kumagai, Megumi Koshiishi, Saori Oishi, Yuki Sueki, Kei Nakajima, Toru Mitsumori, Keita Kirito
Coagulation abnormalities are a rare but critical complication associated with plasma cell diseases. We herein present a case of multiple myeloma (MM) with complicated coagulopathy. Initially, the patient showed severe bleeding tendency due to concomitant acquired hemophilia A and acquired von Willebrand syndrome. Interestingly, the patient also exhibited hyperactivation of factor IX. During treatment for MM, the bleeding complications were ameliorated; however, the patient had central retinal vein occlusion...
July 6, 2018: Internal Medicine
Inna Tzoran, Ron Hoffman, Manuel Monreal
There is a growing proportion of the elderly population in the Western world, and these individuals require special considerations regarding a broad variety of aspects, including treatment approaches to illnesses that affect all age groups. The hemostatic system in individuals changes considerably with aging. Specifically, changes in levels of procoagulant and natural anticoagulant factors along with thrombopathy simultaneously create a hypercoagulable state and hemostatic difficulties. Underlying morbidities, such as congestive heart failure, chronic obstructive pulmonary disease, diabetes mellitus, and cancer, increase the risk for venous and arterial thrombosis...
October 2018: Seminars in Thrombosis and Hemostasis
Francesca Stufano, Marco Boscarino, Paolo Bucciarelli, Luciano Baronciani, Alberto Maino, Giovanna Cozzi, Flora Peyvandi
An increased von Willebrand factor propeptide (VWFpp) to VWF antigen (VWF:Ag) ratio (VWFpp/VWF:Ag) indicates an enhanced clearance of VWF. This finding has been described in von Willebrand disease (VWD) and in acquired von Willebrand syndrome (AVWS). A distinction between these two diseases, one congenital and the other acquired, is primarily based on family and personal history of bleeding. However, if this information is scanty, the diagnosis might be challenging due to the lack of an effective diagnostic biomarker...
June 18, 2018: Seminars in Thrombosis and Hemostasis
V Icheva, M Nowak-Machen, U Budde, K Jaschonek, F Neunhoeffer, M Kumpf, M Hofbeck, C Schlensak, G Wiegand
Essentials Bleeding complications during congenital heart disease surgery in neonatal age are very common. We report the perioperative incidence of acquired von Willebrand syndrome (aVWS) in 12 infants. aVWS was detected in 8 out of 12 neonates and infants intraoperatively after cardiopulmonary bypass. Ten patients received von Willebrand factor concentrate intraoperatively and tolerated it well. SUMMARY: Background Cardiac surgery of the newborn and infant with complex congenital heart disease (CHD) is associated with a high rate of intraoperative bleeding complications...
November 2018: Journal of Thrombosis and Haemostasis: JTH
Bérangère S Joly, Paul Coppo, Agnès Veyradier
Child-onset thrombotic thrombocytopenic purpura (TTP) is a rare entity of thrombotic microangiopathy (TMA). The pathophysiology of the disease is based on a severe functional deficiency of ADAMTS13 (activity <10%), the specific von Willebrand factor (VWF)-cleavage protease. This deficiency may be either acquired (associated anti-ADAMTS13 autoantibodies) or congenital (resulting from biallelic mutations of ADAMTS13 gene). ADAMTS13 deficiency is responsible for the accumulation of high molecular weight multimers of VWF and the formation of platelet thrombi in the microcirculation...
October 2018: European Journal of Haematology
Anna M Randi, Koval E Smith, Giancarlo Castaman
Several important physiological processes, from permeability to inflammation to hemostasis, take place at the vessel wall and are regulated by endothelial cells (ECs). Thus, proteins that have been identified as regulators of one process are increasingly found to be involved in other vascular functions. Such is the case for von Willebrand factor (VWF), a large glycoprotein best known for its critical role in hemostasis. In vitro and in vivo studies have shown that lack of VWF causes enhanced vascularization, both constitutively and following ischemia...
July 12, 2018: Blood
Honami Kurahashi, Yoshinari Kawabata, Yoshihiro Michishita, Atsushi Kitabayashi, Takahiro Kobayashi, Akihiro Kitadate, Naoto Takahashi
A 61-year-old female with no history of bleeding was admitted to our hospital owing to persistent bleeding after the left knee joint injection and activated partial thromboplastin time prolongation. Subsequent coagulation tests revealed a critically declined level of the von Willebrand factor (VWF) antigen (<10%) and activity (<10%) measurement besides a significantly declined factor VIII activity (4%). Despite diagnosing her with acquired von Willebrand syndrome (AvWS) and managing her bleeding with desmopressin acetate hydrate (DDAVP), we could not precisely make a definitive diagnosis the underlying disorder...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
C P M Hayward
INTRODUCTION: Laboratory investigations for bleeding disorders are warranted when an individual has a personal and/or family history of bleeding, and/or laboratory findings that suggest the possibility of an inherited or acquired bleeding disorder. METHODS: This review summarizes author's experience with ordering and reporting on diagnostic investigations for common and rare bleeding disorders, with consideration of recent articles on diagnosing bleeding disorders...
May 2018: International Journal of Laboratory Hematology
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