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Von willebrands disease

Hanny Al-Samkari, Rachael F Grace, Jean M Connors
Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder resulting in potentially life-threating systemic thrombotic microangiopathy due to production of antibodies directed against the von Willebrand factor-cleaving protease ADAMTS13. Typically managed with plasma exchange, glucocorticoids, and the first-generation anti-CD20 monoclonal antibody rituximab, patients with multiple relapses or refractory disease present unique management challenges. We describe a case of a young woman with multiple relapses of TTP despite standard therapy who was treated with ofatumumab, a second-generation anti-CD20 monoclonal antibody, after developing a severe hypersensitivity reaction to rituximab precluding its use...
March 21, 2018: Journal of Thrombosis and Thrombolysis
Y Feng, S Y Mei, N Liu, R X Guo
No abstract text is available yet for this article.
March 14, 2018: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Katrine Munk Begtrup, Johanne Andersen Hojbjerg, Birgitte Vrou Offersen, Anne-Mette Hvas
Cancer is associated with increased risk of venous thromboembolic disease. Venous thromboembolic disease accounts for a substantial addition to morbidity and mortality rates in cancer patients and is the second leading cause of death in cancer patients, exceeded only by the underlying cancer. Only few previous studies have investigated the influence of radiotherapy on hemostasis and whether radiotherapy in itself causes an increased risk of venous thromboembolic disease. The aim was to investigate if adjuvant radiotherapy affects hemostasis after surgery and chemotherapy in patients with breast cancer...
March 19, 2018: Platelets
Federico Moccetti, Craig C Weinkauf, Brian P Davidson, J Todd Belcik, Edmund R Marinelli, Evan Unger, Jonathan R Lindner
The aim of this study was to evaluate a panel of endothelium-targeted microbubble (MB) ultrasound contrast agents bearing small peptide ligands as a human-ready approach for molecular imaging of markers of high-risk atherosclerotic plaque. Small peptide ligands with established affinity for human P-selectin, VCAM-1, LOX-1 and von Willebrand factor (VWF) were conjugated to the surface of lipid-stabilized MBs. Contrast-enhanced ultrasound (CEUS) molecular imaging of the thoracic aorta was performed in wild-type and gene-targeted mice with advanced atherosclerosis (DKO)...
March 13, 2018: Ultrasound in Medicine & Biology
Cédric Garcia, Nicolas Montée, Julien Faccini, Jennifer Series, Olivier Meilhac, Anne-Valérie Cantero, Pauline Le Faouder, Meyer Elbaz, Bernard Payrastre, Cécile Vindis
BACKGROUND: While high-density lipoproteins (HDL) have anti-thrombotic effects by reducing platelet activation, the relationship between HDL levels and the risk of acute coronary syndrome (ACS) is unclear since HDL particles are heterogeneous in composition and biological properties OBJECTIVE: We characterized the effects of HDL2 and HDL3 subclasses from ACS patients and non-coronary artery disease (CAD) subjects on platelet activation. METHODS: We measured platelet aggregation and ex vivo thrombus formation, analyzed signaling pathways by flow cytometry and performed a targeted lipidomics analysis on HDL subclasses RESULTS: Analysis of human platelet aggregation in suspension, adhesion on von Willebrand factor and thrombus formation on collagen under arterial shear demonstrated that HDL2 subclass from ACS patients had a higher antiplatelet potency compared to ACS HDL3 particles and HDL subclasses from non-CAD subjects...
March 15, 2018: Journal of Thrombosis and Haemostasis: JTH
Yuka Matsunaga, Masataka Ishimura, Hazumu Nagata, Kiyoshi Uike, Tadamune Kinjo, Masayuki Ochiai, Kenichiro Yamamura, Hidetoshi Takada, Yoshihisa Tanoue, Masaki Hayakawa, Masanori Matsumoto, Toshiro Hara, Shouichi Ohga
BACKGROUND: Thrombotic microangiopathies (TMA) are microvascular occlusive disorders characterized by systemic or intrarenal platelet aggregation, thrombocytopenia, and red cell fragmentation. Post-operative TMA mostly occurs in adult patients with cardiovascular surgery, with the distinct pathophysiology from classical thrombotic thrombocytopenic purpura (TTP) although the exact pathophysiology remains unclear. CASE PRESENTATION: A one-month-old infant developed TMA after the initial surgery of double outlet right ventricle...
February 7, 2018: Pediatrics and Neonatology
Qizhen Shi
Gene therapy is an attractive approach for disease treatment. Since platelets are abundant cells circulating in blood with the distinctive abilities of storage and delivery and fundamental roles in hemostasis and immunity, they could be a unique target for gene therapy of diseases. Recent studies have demonstrated that ectopic expression of factor VIII (FVIII) in platelets under control of the platelet-specific promoter results in FVIII storage together with its carrier protein von Willebrand factor (VWF) in α-granules and the phenotypic correction of hemophilia A...
June 15, 2018: Molecular Therapy. Methods & Clinical Development
Alfia Khaibullina, Elena A Adjei, Nowah Afangbedji, Andrey Ivanov, Namita Kumari, Luis E F Almeida, Zenaide M N Quezado, Sergei Nekhai, Marina Jerebtsova
Sickle cell disease patients are at increased risk of developing a chronic kidney disease. Endothelial dysfunction and inflammation associated with hemolysis lead to vasculopathy and contribute to the development of renal disease. Here we used a Townes sickle cell disease mouse model to examine renal endothelial injury. Renal disease in Townes mice was associated with glomerular hypertrophy, capillary dilation and congestion, and significant endothelial injury. We also detected substantial renal macrophage infiltration, and accumulation of macrophage stimulating protein 1 in glomerular capillary...
March 8, 2018: Haematologica
J G Brunet, J K Iyer, M S Badin, L Graf, K A Moffat, M Timleck, E Spitzer, C P M Hayward
INTRODUCTION: Dense granule (DG) deficiency (DGD) is a feature of some platelet function disorders (PFD) with a prevalence similar to von Willebrand disease. Most laboratories assess for DGD using whole mount platelet preparations and electron microscopy (EM). We evaluated our experiences with this test and associations between DGD and bleeding. METHODS: Dense granule EM records for 2006-2017 were examined for patients and simultaneously tested controls, and for an overlapping PFD study cohort to evaluate findings and their relationship to bleeding...
March 6, 2018: International Journal of Laboratory Hematology
Koki Sato, Shintaro Kuroda, Tsuyoshi Kobayashi, Seiichi Shimizu, Masahiro Ohira, Hiroyuki Tahara, Kentaro Ide, Teruhisa Fujii, Hideki Ohdan
INTRODUCTION: Although von Willebrand disease (VWD) is a common inherited bleeding disorder, very few cases of surgery in patients with VWD have been reported. PRESENTATION OF CASE: A 77-year-old man was referred to our hospital for treatment of hepatocellular carcinoma (HCC) based on type C chronic hepatitis. He had also been treated for VWD in the hematology department of another hospital. Partial hepatectomy was performed with the administration of factor VIII/von Willebrand factor concentrate just before and after the operation...
February 24, 2018: International Journal of Surgery Case Reports
Mathilde Fénelon, Sabine Castet, Jean-Christophe Fricain, Sylvain Catros
Introduction: Von Willebrand Disease is the most common inherited bleeding disorder. In the general population, 1/8000 patients are affected. Primary hemostasis (platelet adhesion) and coagulation (protection of Factor VIII) are altered. Among several bleeding symptoms, these patients suffer from excessive bleeding of oral mucosa and dental management requires a close collaboration between haematologists and oral surgeons. Materials & Methods: Guided implant surgery can be used to increase the accuracy of implant placement and to reduce the overall morbidity of this surgical procedure by using a flapless surgery technique...
2018: Open Dentistry Journal
Johan Ljungberg, Mikael Janiec, Ingvar A Bergdahl, Anders Holmgren, Johan Hultdin, Bengt Johansson, Ulf Näslund, Agneta Siegbahn, Tove Fall, Stefan Söderberg
Background -Aortic valve stenosis (AS) is the most common indication for cardiac valve surgery; untreated AS is linked to high mortality. The aetiological background of AS is unknown. Previous human studies were typically based on case-control studies. Biomarkers identified in prospective studies could lead to novel mechanistic insights. Methods -Within a large population survey with blood samples obtained at baseline, 334 patients were identified that later underwent surgery for AS (median age (interquartile range) 59...
February 27, 2018: Circulation
Marika Pikta, Galina Zemtsovskaja, Hector Bautista, Georges Nouadje, Timea Szanto, Margus Viigimaa, Valdas Banys
BACKGROUND: The von Willebrand factor (VWF) multimer test is required to correctly subtype qualitative type 2 von Willebrand disease (VWD). The current VWF multimer assays are difficult, nonstandardized, and time-consuming. The purpose of this study was to evaluate the clinical utility of the commercial VWF multimer kit by Sebia (Lisses, France), an electrophoresis technique yielding same-day results. METHODS: Ten healthy volunteer plasma samples, in-house reference plasma (IRP) and commercial normal plasma (CNP) samples, 10 plasma samples from patients with a known VWD type, 1 hemophilia A plasma sample, and 7 external quality assurance (EQA) samples were analyzed using the commercial VWF multimer kit...
February 17, 2018: Journal of Clinical Laboratory Analysis
Christine Demers, Christine Derzko, Michele David, Joanne Douglas
OBJECTIVE: The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in adolescents presenting with menorrhagia. OPTIONS: Diagnostic tools and specific medical and, where appropriate, surgical alternatives to management are reviewed and evidence-based recommendations presented. EVIDENCE: A MEDLINE search of the English literature between January 1975 and November 2003 was performed using the following key words: menorrhagia, uterine bleeding, pregnancy, von Willebrand, congenital bleeding disorder, desmopressin/DDAVP, tranexamic acid, oral contraceptives, medroxyprogesterone, therapy, hysterectomy, anesthesia, epidural, spinal...
February 2018: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
N V Khatri, B Patel, D R Kohli, S S Solomon, K Bull-Henry, C M Kessler
INTRODUCTION: Lenalidomide is a thalidomide analog with anti-angiogenic properties. Previous case reports suggest its efficacy in preventing gastrointestinal bleeding (GIB) secondary to angiodysplasia (AD) in hereditary haemorrhagic telangiectasia and potentially in reversing AD. We present the first case series to explore lenalidomide as a treatment for AD-related GIB in patients with von Willebrand disease (VWD). METHODS: A retrospective chart review was conducted to include patients with VWD, who were evaluated from 2010 to 2013 and who had received lenalidomide to treat recurrent GIB secondary to AD...
February 15, 2018: Haemophilia: the Official Journal of the World Federation of Hemophilia
Marina Panova-Noeva, Andreas Schulz, Natalie Arnold, Iris M Hermanns, Jürgen H Prochaska, Dagmar Laubert-Reh, Henri M Spronk, Maria Blettner, Manfred Beutel, Norbert Pfeiffer, Thomas Münzel, Karl J Lackner, Hugo Ten Cate, Philipp S Wild
BACKGROUND: The advancements in cancer treatment and detection of early cancer have resulted in steady increase of cancer survivors over the years. However, due to long term toxic effects of chemotherapy and radiotherapy, the incidence of cardiovascular disease (CVD) is increasing in survivors. OBJECTIVES: This study investigates traditional cardiovascular risk factors (CVRFs), inflammation and coagulation profile in long-term cancer survivors (cancer diagnosis ≥ five years) from a large adult population-based study sample...
February 12, 2018: Journal of Thrombosis and Haemostasis: JTH
Nicholas Bercovici, Kristen Russomanno, Frank Santoro, Christopher Scola, Michael Murphy, Zendee Elaba
Injection drug abuse (IDA) is known to cause a spectrum of systemic and cutaneous complications. Despite the increasing incidence of IDA around the world, there is a paucity of literature discussing cutaneous complications from a dermatopathologic perspective. We present a case of a 35-year-old male with a complex medical history of Von Willebrand disease, Behcet's disease and diverticular disease. Following a sigmoidectomy/colostomy for diverticular perforation, he presented with fever and an indurated right arm displaying livedoid purpura...
February 12, 2018: Journal of Cutaneous Pathology
V Nummi, R Lassila, L Joutsi-Korhonen, E Armstrong, T Szanto
INTRODUCTION: Diagnosis of von Willebrand disease (VWD) is challenging, particularly for type 1. The current diagnostic guidelines emphasize simultaneous bleeding symptoms and von Willebrand factor (VWF) levels of <30-40 IU/dL. Historical diagnoses require updated evaluation. We assessed the accuracy of past VWD diagnoses in our comprehensive care center with the standardized bleeding score (BS) and central laboratory analysis, focusing on VWF-dependent platelet functions in whole blood...
February 10, 2018: International Journal of Laboratory Hematology
Karl C Desch
Von Willebrand factor (VWF) is a multimeric plasma glycoprotein that plays a central role in the initiation of blood coagulation. Through interactions between its specific functional domains, the vascular wall, coagulation factor VIII, and platelet receptors, VWF maintains hemostasis by binding to platelets and delivering factor VIII to the sites of vascular injury. In the healthy human population, plasma VWF levels vary widely. The important role of VWF is illustrated by individuals at the extremes of the normal distribution of plasma VWF concentrations where individuals with low VWF levels are more likely to present with mucocutaneous bleeding...
2018: F1000Research
Hong Jin, Yifei Chen, Bilei Wang, Yi Zhu, Long Chen, Xiqiong Han, Genshan Ma, Naifeng Liu
BACKGROUND: Brain-derived neurotrophic factor (BDNF) is a neurotrophin involved in angiogenesis and maintenance of endothelial integrity. Whether circulating BDNF levels are associated with von Willebrand factor (vWF) levels, which are indicators of endothelial dysfunction is not known. This study investigated the association between plasma BNDF and vWF levels and whether these biomarkers could predict cardiovascular events at a 12-month follow-up in patients with stable coronary artery disease (CAD)...
February 6, 2018: BMC Cardiovascular Disorders
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