Read by QxMD icon Read

acquired von willebrand

Alessandra Molteni, Zubair Ph Masri, Kenny Wq Low, Haitham N Yousef, Johann Sienz, Katharine H Fraser
Ventricular assist devices have become the standard therapy for end-stage heart failure. However, their use is still associated with severe adverse events related to the damage done to the blood by fluid dynamic stresses. This damage relates to both the stress magnitude and the length of time the blood is exposed to that stress. We created a dye washout technique which combines experimental and numerical approaches to measure the washout times of ventricular assist devices. The technique was used to investigate washout characteristics of three commercially available and clinically used ventricular assist devices: the CentriMag, HVAD and HeartMate II...
March 1, 2018: International Journal of Artificial Organs
C Gebhard, F Maafi, B E Stähli, A Bonnefoy, C E Gebhard, W Nachar, A Benjamim de Oliveira Moraes, M Mecteau, T Mihalache-Avram, V Lavoie, A E Kernaleguen, Y Shi, D Busseuil, M Chabot-Blanchet, L P Perrault, D Rhainds, E Rhéaume, J C Tardif
BACKGROUND: Infusions of apolipoprotein A-I (apoA-I), the major protein component of high-density lipoproteins (HDL), result in aortic valve stenosis (AVS) regression in experimental models. Severe AVS can be complicated by acquired von Willebrand syndrome, a haemorrhagic disorder associated with loss of high-molecular-weight von Willebrand factor (vWF) multimers (HMWM), the latter being a consequence of increased shear stress and enhanced vWF-cleaving protease (ADAMTS-13) activity. Although antithrombotic actions of HDL have been described, its effects on ADAMTS-13 and vWF in AVS are unknown...
February 2018: Thrombosis and Haemostasis
Tara L Molina, Jill Krisl, Kevin Donahue, Sara Varnado
Left ventricular assist devices (LVADs) offer a therapeutic strategy for patients with end-stage heart failure. Increased device utilization has also increased the incidence of device-related complications including gastrointestinal bleeding (GIB). Multiple mechanisms have been proposed in the pathophysiology of continuous-flow LVAD-associated GIB including physiologic changes associated with high shear and nonpulsatile flow such as gastrointestinal arteriovenous malformations and acquired von Willebrand syndrome...
February 5, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Candice M Baldeo, Candido E Rivera, Han W Tun, Prakash Vishnu
von Willebrand disease (VWD) is a common bleeding disorder caused by defective or low levels of von Willebrand factor (VWF). Although most cases of VWD are caused by genetic mutations, some are acquired due to various disease states. In managing VWD, the aim is to normalize plasma levels of both VWF and factor VIII (FVIII), as this aids in hemostasis. Desmopressin usually corrects VWF level in type 1 VWD by inducing the release of endogenous VWF. In cases where desmopressin is ineffective or cannot be used, transfusion of virally inactivated, plasma-derived VWF/FVIII concentrate or infusion of recombinant VWF (Vonvendi) is indicated...
2018: Journal of Blood Medicine
Joseph L Blackshear
Gastrointestinal bleeding with severe aortic stenosis was originally described in the 1950s by Heyde, although for years, the association was debated. Further discovery of mechanisms and the ubiquity and severity of acquired von Willebrand syndrome in the left ventricular assist device therapy have removed any doubts. At this time, gastrointestinal bleeding from intestinal angiodysplasia in patients with turbulence-related proteolysis of the highest molecular weight multimers of von Willebrand factor is now known to occur in patients with aortic stenosis, and also subaortic obstruction and associated mitral insufficiency in hypertrophic cardiomyopathy, isolated mitral and aortic insufficiency, endocarditis, and in patients with prosthetic valve dysfunction, either from stenosis or insufficiency...
February 3, 2018: Current Treatment Options in Cardiovascular Medicine
Valter Romão de Souza, Ana Beatriz Cavalcante de Oliveira, Ana Maria Vanderlei, Amanda Queiroz da Mota Silveira Aroucha, Bruna Pontes Duarte, Aureli Nunes Machado, Lívia Netto Chaer, Cláudia Wanderley de Barros Correia, Maria da Conceição de Barros Correia, Manuela Freire Hazin Costa
BACKGROUND: Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. The diagnosis is challenging due to the nonspecific signs and symptoms, as well as the rarity of the disease...
January 22, 2018: Journal of Medical Case Reports
Uma H Athale, T Mizrahi, C Laverdière, T Nayiager, Y-L Delva, G Foster, L Thabane, M David, J-M Leclerc, A K C Chan
BACKGROUND: Children with acute lymphoblastic leukemia (ALL) have increased risk of thromboembolism (TE). However, the predictors of ALL-associated TE are as yet uncertain. OBJECTIVE: This exploratory, prospective cohort study evaluated the effects of clinical (age, gender, ALL risk group) and laboratory variables (hematological parameters, ABO blood group, inherited and acquired prothrombotic defects [PDs]) at diagnosis on the development of symptomatic TE (sTE) in children (aged 1 to ≤18) treated on the Dana-Farber Cancer Institute ALL 05-001 study...
January 15, 2018: Pediatric Blood & Cancer
Ulrich Geisen, Kerstin Brehm, Georg Trummer, Michael Berchtold-Herz, Claudia Heilmann, Friedhelm Beyersdorf, Johannes Schelling, Axel Schlagenhauf, Barbara Zieger
BACKGROUND: The number of implanted ventricular assist devices (VADs) has increased significantly recently. Bleeding, the most frequent complication, cannot be solely attributed to anticoagulation therapy. Acquired von Willebrand syndrome (AVWS) caused by increased shear stress is frequent in VAD patients and can increase the bleeding risk. The HeartMate III (HM III) is a novel left VAD featuring potential improvements over the HeartMate II. METHODS AND RESULTS: In this study, we investigated the prevalence and onset of AVWS in 198 VAD patients...
January 13, 2018: Journal of the American Heart Association
Hiroaki Tanaka, Yurie Nagai, Chihiro Kuwabara, Ryo Shimizu, Akihide Umeki, Tetsufumi Yamamoto
Acquired von Willebrand syndrome (AVWS) is a bleeding disorder caused by an acquired deficiency of von Willebrand factor (vWF). Some patients with AVWS show a low bleeding tendency and are diagnosed by the presence of a mild prolongation of activated partial thromboplastin time (APTT) preoperatively. Another cause of APTT prolongation is the presence of antiphospholipid antibody (aPL). We experienced a case of AVWS due to aortic valve stenosis in a patient with aPL in whom aortic valve replacement surgery was successful with vWF replacement...
January 11, 2018: Internal Medicine
Bogac Ercig, Kanin Wichapong, Chris P M Reutelingsperger, Karen Vanhoorelbeke, Jan Voorberg, Gerry A F Nicolaes
ADAMTS13 (A D: isintegrin A: nd M: etalloprotease with a T: hromboS: pondin type-1 motif, member 13: ) and von Willebrand factor (VWF) can be considered as scale weights which control platelet adhesion during primary haemostasis. In a very uncommon condition designated thrombotic thrombocytopenic purpura (TTP), functional absence of ADAMTS13 tips the balance toward VWF-mediated platelet adhesion in the microcirculation. TTP is associated with a high mortality and arises from either a congenital or acquired autoimmune deficiency of the plasma enzyme ADAMTS13...
January 2018: Thrombosis and Haemostasis
R Muslem, K Caliskan, F W G Leebeek
Chronic heart failure (HF) is a major emerging health care problem, associated with a high morbidity and mortality. Left ventricular assist devices (LVADs) have emerged as a successful treatment option for patients with end-stage HF. Despite its great benefit, the use of LVAD is associated with a high risk for complications. Bleeding, pump thrombosis, and thrombo-embolic events are frequently observed complications, with bleeding complications occurring in over a third of the patients. Although the design of the third generation LVAD has improved greatly, these hemostatic complications still occur...
December 23, 2017: Journal of Thrombosis and Haemostasis: JTH
Amihai Rottenstreich, Ela Shai, Geffen Kleinstern, Galia Spectre, David Varon, Yosef Kalish
OBJECTIVE: We aimed to determine hemostatic changes and characterize the procoagulant potential among reactive thrombocytosis (RT) patients. METHODS: Sixty RT patients (median platelet count 718X109 /L) and 20 healthy persons were tested for complete blood count, C-reactive protein, von Willebrand factor (VWF), factor VIII and fibrinogen, and thrombin generation. Platelet studies, including light transmission aggregometry and Cone and Plate(let) Analyzer, were also conducted...
December 14, 2017: European Journal of Haematology
Rashad Zayat, Mohammad Amen Khattab, Oliver Grottke, Markus Honickel, Andreas Goetzenich, Ajay Moza, Christian Stoppe, Rüdiger Autschbach, Lachmandath Tewarie
BACKGROUND: In long-term left ventricular assist device (LVAD) therapy, recurrent bleeding events may justify cessation of anticoagulation therapy (AT). However, data about THE safety and risks of AT cessation in LVAD patients are scarce.Methods and Results:Between 2010 and 2015, 128 patients received a HeartMate II (HMII). Following recurrent bleeding events, we ceased vitamin K antagonist (VKA) therapy in 13 patients (10%) (no-VKA group). To characterize the hemostatic profile, we performed von Willebrand factor (vWF), platelet function (PF), and other hemostatic tests in all HMII patients...
December 12, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
Oliver Andres, Katja Henning, Gabriele Strauß, Annerose Pflug, Georgi Manukjan, Harald Schulze
A high proportion of patients with mucocutaneous bleeding diathesis and suspected inherited or acquired platelet disorder remain without diagnosis even after comprehensive laboratory testing. Since flow cytometry allows investigation of resting and activated platelets on the single cell level by requiring only minimal amounts of blood, this method has become an important assay within the diagnostic algorithm, especially in pediatrics. We therefore developed a standardized and modular flow cytometric approach that contributes to clarify impaired platelet function in a rational step-by-step manner...
December 11, 2017: Platelets
Sijan Basnet, Catherine Lin, Rashmi Dhital, Izza Mir, Elan Mohanty, Biswaraj Tharu, Sushil Ghimire, Dilli Ram Poudel
We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor assay, and negative Bethesda assay, indicating acquired von Willebrand disease. Immunofluorescence to find an underlying etiology was suggestive of MGUS...
2017: Case Reports in Oncological Medicine
Joerg Kellermair, Helmut W Ott, Michael Spannagl, Josef Tomasits, Juergen Kammler, Hermann Blessberger, Christian Reiter, Clemens Steinwender
Acquired von Willebrand syndrome (AVWS) associated with severe aortic stenosis (AS) has been frequently subclassified into a subtype 2A based on the deficiency of high-molecular-weight (HMW) multimers as it is seen in inherited von Willebrand disease (VWD) type 2A. However, the multimeric phenotype of VWD type 2A does not only include an HMW deficiency but also a decrease in intermediate-molecular-weight (IMW) multimers and an abnormal inner triplet band pattern. These additional characteristics have not been evaluated in AVWS associated with severe AS...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
Christina Hellmann, Axel Schmutz, Johannes Kalbhenn
INTRODUCTION: Veno-venous extracorporeal membrane oxygenation (vvECMO) used for respiratory support is associated with clinical bleeding in at least one third of patients. Mechanisms promoting bleeding, like acquired von Willebrand syndrome, cannot be identified by routine coagulation tests. This study was performed to evaluate rotational Thrombelastography (ROTEM™) for specific results predicting bleeding events during vvECMO. METHODS: Five hundred and thirty-four ROTEM™ analyses of 57 patients over 574 days have been evaluated...
November 1, 2017: Perfusion
Jamie M O'Sullivan, Roger J S Preston, Tracy Robson, James S O'Donnell
von Willebrand factor (VWF) is a complex multimeric plasma glycoprotein that plays critical roles in normal hemostasis. However, additional novel roles for VWF in modulating cancer cell biology, and in particular tumor metastasis, have recently been reported. Markedly elevated plasma VWF levels were associated with advanced tumor stage and metastatic disease. These observations have raised the question of whether VWF may be involved in regulating tumor progression. Interestingly, novel findings indicate that VWF is expressed by a variety of tumor cells of nonendothelial origin...
November 17, 2017: Seminars in Thrombosis and Hemostasis
Iki Adachi, Vadim Kostousov, Lisa Hensch, Martin A Chacon-Portillo, Jun Teruya
Ventricular-assist devices (VADs) have seen increased utilization in the pediatric population. Formerly, this therapeutic modality was limited to only the pulsatile VAD, EXCOR (Berlin Heart GmbH). However, the continuous flow VAD devices, HeartMate II (Abbott Inc.) and HeartWare (Medtronic Inc.), are now increasingly used in this population. Postoperatively, VAD patients are acutely anticoagulated using unfractionated heparin, often beginning 24 to 48 hours after VAD placement. Once the patient is stabilized and ready to transition to a lower acuity or outpatient setting, low-molecular-weight heparin or warfarin therapy may be instituted...
February 2018: Seminars in Thrombosis and Hemostasis
Clinton D Cochran, Rohit S Madani, Daniel Peltier, Steven Pipe, Sonal T Owens
An infant with coarctation of the aorta and Williams syndrome was noted to have petechiae in cardiology clinic prior to planned surgical intervention. Workup revealed acquired von Willebrand syndrome secondary to the high shear force generated by the aortic coarctation. He was treated with intra- and postoperative Humate P; there were no postoperative bleeding complications. His acquired von Willebrand syndrome resolved postoperatively.
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"