12,312 results match your criteria von Willebrand Disease


A Case of Acquired von Willebrand Disease Secondary to Myeloproliferative Neoplasm.

Case Rep Oncol 2020 May-Aug;13(2):733-737. Epub 2020 Jun 26.

Department of Hematology, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

Acquired von Willebrand Disease (AVWD) is a rare disorder in which qualitative or quantitative defects in von Willebrand factor (VWF) occur secondary to other conditions. AVWD occurs in patients with myeloproliferative disorders due to formation of autoantibodies against VWF and development of excessive shear stress causing disruption of VWF multimers. AVWD is different from congenital VWD in its acute onset and absence of family history. Read More

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http://dx.doi.org/10.1159/000507883DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7383181PMC

Hemorrhagic Synovitis of the First Metatarsophalangeal Joint: A Case Report.

JBJS Case Connect 2020 Jul-Sep;10(3):e2000168

1Department of Orthopedic Surgery, University of Kansas Medical Center Kansas City, Kansas 2Department of Pathology, University of Kansas Medical Center, Kansas City, Kansas.

Case: A 69-year-old woman presented with a painful mass at her first metatarsophalangeal joint. Further evaluation was concerning for a neoplastic process, leading to surgical intervention. Pathological examination demonstrated hemosiderotic synovitis, and hematologic evaluation led to a new diagnosis of von Willebrand disease. Read More

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http://dx.doi.org/10.2106/JBJS.CC.20.00168DOI Listing

Novel Approaches to Fine-Tune Therapeutic Targeting of Platelets in Atherosclerosis: A Critical Appraisal.

Thromb Haemost 2020 Aug 9. Epub 2020 Aug 9.

Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK) e.V., Partner Site Munich Heart Alliance, Munich, Germany.

The pathogenesis of atherosclerotic vascular disease is driven by a multitude of risk factors intertwining metabolic and inflammatory pathways. Increasing knowledge about platelet biology sheds light on how platelets take part in these processes from early to later stages of plaque development. Recent insights from experimental studies and mouse models substantiate platelets as initiators and amplifiers in atherogenic leukocyte recruitment. Read More

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http://dx.doi.org/10.1055/s-0040-1714352DOI Listing

VWF/ADAMTS13 imbalance, but not global coagulation or fibrinolysis, is associated with outcome and bleeding in acute liver failure.

Hepatology 2020 Aug 7. Epub 2020 Aug 7.

Surgical Research Laboratory, Department of Surgery, University of Groningen, University Medical Center Groningen, Groningen, the Netherlands.

Background And Aims: Recent studies of acute liver failure (ALF) in man and animals have suggested that rebalanced hemostasis occurs, with distinct hypercoagulable features clinically evidenced by a low risk of bleeding. Rodent models have shown a link between intrahepatic microthrombus formation and progression of ALF. We sought to confirm these earlier findings in a large series of well-characterized ALF patients from the Acute Liver Failure Study Group (ALFSG). Read More

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http://dx.doi.org/10.1002/hep.31507DOI Listing

[Acquired von Willebrand syndrome].

Rinsho Ketsueki 2020 ;61(7):809-817

Department of Transfusion Medicine, Nara Medical University.

Von Willebrand disease (VWD) is among the most common inherited bleeding disorders. Interestingly, acquired von Willebrand syndrome (AVWS) is diagnosed much less frequently, but can be identified in association with a substantial number of medical conditions and diseases, including lymphoproliferative (48%), cardiovascular (21%), myeloproliferative (15%), other neoplastic (5%), and autoimmune disorders (2%). Most recently, AVWS has been diagnosed in patients with aortic valve stenosis (AS, 79%) and continuous-flow left ventricular assist devices (LVAD, up to 100%). Read More

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http://dx.doi.org/10.11406/rinketsu.61.809DOI Listing
January 2020

[Acquired coagulation disorders].

Authors:
Nobuaki Suzuki

Rinsho Ketsueki 2020 ;61(7):779-784

Department of Transfusion Medicine, Nagoya University Hospital.

Acquired coagulation inhibitors have become a popular area of research because they cause severe bleeding tendency in many patients. The use of acquired coagulation inhibitors requires rapid and precise diagnosis. Some acquired coagulation inhibitors show prolongation in the activated partial thromboplastin time (APTT) and/or prothrombin time (PT). Read More

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http://dx.doi.org/10.11406/rinketsu.61.779DOI Listing
January 2020

Acute exacerbations of COPD are associated with a prothrombotic state through platelet-monocyte complexes, endothelial activation and increased thrombin generation.

Respir Med 2020 Jul 25;171:106094. Epub 2020 Jul 25.

Department of Respiratory Medicine, Gelre Ziekenhuizen, Apeldoorn, the Netherlands; Department of Respiratory Medicine, Maastricht University Medical Centre, Maastricht, the Netherlands; NUTRIM School of Nutrition and Translational Research in Metabolism, University of Maastricht, Maastricht, the Netherlands.

Introduction: Patients with chronic obstructive pulmonary disease (COPD) are at increased risk for cardiovascular events, particularly following an acute exacerbation (AE-COPD). Exacerbations are associated with increased systemic inflammation, which may drive coagulation. This prospective cohort study aimed to determine how an AE-COPD affects platelet activation, the endothelium, plasmatic coagulation and fibrinolysis, and its association with systemic inflammation. Read More

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http://dx.doi.org/10.1016/j.rmed.2020.106094DOI Listing
July 2020
3.086 Impact Factor

Unique fibrinogen-binding motifs in the nucleocapsid phosphoprotein of SARS CoV-2: Potential implications in host-pathogen interactions.

Authors:
Nikhil Sangith

Med Hypotheses 2020 Jun 24;144:110030. Epub 2020 Jun 24.

Xact Diagnotek Private Limited, Anna University Bioincubator, Taramani, Chennai 600113, India. Electronic address:

Novel Coronavirus (SARS CoV-2), the etiological agent for the highly contagious Corona virus disease-2019 (COVID-19) pandemic has threatened global health and economy infecting around 5.8 million people and causing over 359,200 deaths (as of 28th May 2020, https://www.worldometers. Read More

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http://dx.doi.org/10.1016/j.mehy.2020.110030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313483PMC

Treatment of acquired thrombotic thrombocytopenic purpura without plasma exchange in selected patients under caplacizumab.

J Thromb Haemost 2020 Aug 5. Epub 2020 Aug 5.

Department of Haemostaseology and Haemophilia Center, University Hospital Frankfurt, Frankfurt am Main, Germany.

Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy. Current standard of care is therapeutic plasma exchange, immunosuppression, and caplacizumab, an anti-von Willebrand Factor Nanobody, which is effective in treating aTTP episodes.

Patients/methods: Here we report on 7 episodes of aTTP treated without plasma exchange in 6 female patients in Germany and Austria. Read More

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http://dx.doi.org/10.1111/jth.15045DOI Listing

Blood Markers of Portal Hypertension Are Associated with Blood Loss and Transfusion Requirements during Orthotopic Liver Transplantation.

Semin Thromb Hemost 2020 Aug 5. Epub 2020 Aug 5.

Section of Hepatobiliary Surgery and Liver Transplantation, Department of Surgery, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.

There is increasing evidence that portal hypertension plays a major role in bleeding risk during orthotopic liver transplantation (OLT). We investigated the association between preoperative blood levels of von Willebrand factor (VWF) and soluble CD163 (sCD163), which are established markers of portal hypertension, and blood loss and transfusion requirements during OLT. We measured levels of VWF and sCD163 in preoperative serum samples of 168 adult patients undergoing a primary OLT between 1998 and 2012. Read More

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http://dx.doi.org/10.1055/s-0040-1714202DOI Listing

Conformation-Dependent Blockage of Activated VWF Improved Outcomes of Traumatic Brain Injury in Mice.

Blood 2020 Aug 5. Epub 2020 Aug 5.

University of Washington, United States.

Traumatic brain injury-induced coagulopathy (TBI-IC) causes life-threatening secondary intracranial bleeding. Its pathogenesis differs mechanistically from that of coagulopathy arising from extracranial injuries and hemorrhagic shock, but it remains poorly understood. We report results of a study designed to test the hypothesis that von Willebrand factor (VWF) released during acute TBI is intrinsically hyperadhesive because its platelet-binding A1-domain is exposed and contributes to TBI-induced vascular leakage and consumptive coagulopathy. Read More

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http://dx.doi.org/10.1182/blood.2020007364DOI Listing

Morphological and Immunohistochemical Description of a Splenic Haemangioma in a Captive European Wolf () and a Review of the Current Literature.

Vet Sci 2020 Aug 3;7(3). Epub 2020 Aug 3.

Department of Comparative Biomedicine and Food Science-University of Padova AGRIPOLIS-Viale dell' Università 16, 35020 Legnaro, Italy.

Neoplastic diseases are rarely described in wild carnivores; only a few reports have been published on this topic. Here, we describe the histological and immunohistochemical features of a haemangioma in the spleen of a grey wolf () and we compare the results with the dog (). Additionally, we list the different publications found in the literature with neoplastic lesions in wolves. Read More

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http://dx.doi.org/10.3390/vetsci7030102DOI Listing

Population Pharmacokinetic Modeling of von Willebrand Factor Activity in von Willebrand Disease Patients after Desmopressin Administration.

Thromb Haemost 2020 Aug 3. Epub 2020 Aug 3.

Hospital Pharmacy-Clinical Pharmacology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Objective:  Most von Willebrand disease (VWD) patients can be treated with desmopressin during bleeding or surgery. Large interpatient variability is observed in von Willebrand factor (VWF) activity levels after desmopressin administration. The aim of this study was to develop a pharmacokinetic (PK) model to describe, quantify, and explain this variability. Read More

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http://dx.doi.org/10.1055/s-0040-1714349DOI Listing

New Onset of Linear Purpura on the Back: Coining Therapy-Associated Ecchymoses.

Cureus 2020 Jun 25;12(6):e8833. Epub 2020 Jun 25.

Dermatology, San Diego Family Dermatology, National City, USA.

Coining therapy is a treatment commonly used in complementary and alternative medicine. The practice has its origins in several different Asian countries. It is used to treat numerous conditions, such as chronic pain, fever, flu, headaches, heatstroke, and upper respiratory infections. Read More

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http://dx.doi.org/10.7759/cureus.8833DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7384708PMC

Insights into ADAMTS13 structure: impact on thrombotic thrombocytopenic purpura diagnosis and management.

Curr Opin Hematol 2020 Sep;27(5):320-326

Laboratory for Thrombosis Research, IRF Life Sciences, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.

Purpose Of Review: Fundamental knowledge on the role of a disintegrin and metalloprotease with thrombospondin type one repeats, member 13 (ADAMTS13) has been crucial to better understand the pathophysiology of the rare and life-threatening disease thrombotic thrombocytopenic purpura (TTP).

Recent Findings: ADAMTS13 works through a molecular zipper mechanism to proteolyze its substrate von Willebrand factor (VWF). Recent insights into the structure and function of ADAMTS13 led to the identification of an allosteric activation mechanism. Read More

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http://dx.doi.org/10.1097/MOH.0000000000000602DOI Listing
September 2020

Endotrophin, an extracellular hormone, in combination with neoepitope markers of von Willebrand factor improves prediction of mortality in the ECLIPSE COPD cohort.

Respir Res 2020 Jul 30;21(1):202. Epub 2020 Jul 30.

Nordic Bioscience A/S, Herlev, Denmark.

Background: Lung epithelial damage, activation of the wound healing cascade, and remodeling of the extracellular matrix (ECM) play a major role in chronic obstructive pulmonary disease (COPD). The pro-peptide of type VI collagen has been identified as the hormone endotrophin. Endotrophin has been shown to promote fibrosis and inflammation, whereas von Willebrand factor (VWF) is a crucial part of wound healing initiation. Read More

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http://dx.doi.org/10.1186/s12931-020-01461-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7393910PMC

[Inherited bleeding disorders in adolescents with excessive menstrual bleeding. Should we evaluate the fibrinolytic pathway?]

Rev Chil Pediatr 2020 Jun;91(3):385-390

Departamento de Pediatría, Pontificia Universidad Católica de Chile, Santiago, Chile.

Introduction: Heavy Menstrual Bleeding (EMB) is a frequent problem in adolescence. The prevalence of inherited bleeding disorders (IBD) as a cause of EMB is not well established and the involvement of fibri nolytic pathway defects has been poorly explored.

Objective: To determine the prevalence of IBD and fibrinolysis defects in adolescents with EMBs. Read More

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http://dx.doi.org/10.32641/rchped.v91i3.1571DOI Listing

Noncanonical type 2B von Willebrand disease associated with mutations in the VWF D'D3 and D4 domains.

Blood Adv 2020 Jul;4(14):3405-3415

Dipartimento di Medicina e Chirurgia Traslazionale, Facoltà di Medicina e Chirurgia Agostino Gemelli, Università Cattolica S. Cuore, Rome, Italy.

We observed a 55-year-old Italian man who presented with mucosal and cutaneous bleeding. Results of his blood analysis showed low levels of von Willebrand factor (VWF) antigen and VWF activity (both VWF ristocetin cofactor and VWF collagen binding), mild thrombocytopenia, increased ristocetin-induced platelet aggregation, and a deficiency of high-molecular-weight multimers, all typical phenotypic hallmarks of type 2B von Willebrand disease (VWD). The analysis of the VWF gene sequence revealed heterozygous in cis mutations: (1) c. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020002334DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391138PMC

Structure of a collagen VI α3 chain VWA domain array: adaptability and functional implications of myopathy causing mutations.

J Biol Chem 2020 Jul 21. Epub 2020 Jul 21.

University of Cologne, Germany.

Collagen VI is a ubiquitous heterotrimeric protein of the extracellular matrix (ECM) that plays an essential role in the proper maintenance of skeletal muscle. Mutations in collagen VI lead to a spectrum of congenital myopathies, from the mild Bethlem Myopathy to the severe Ullrich Congenital Muscular Dystrophy. Collagen VI contains only a short triple helix and consists primarily of von Willebrand Factor type A (VWA) domains, protein-protein interaction modules found in a range of ECM proteins. Read More

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http://dx.doi.org/10.1074/jbc.RA120.014865DOI Listing

Pre-surgical level of von Willebrand factor as an evident indicator of breast cancer recurrence.

Cancer Biomark 2020 Jul 17. Epub 2020 Jul 17.

Department of Pathophysiology, Faculty of Pharmacy, Nicolaus Copernicus University, Collegium Medicum in Bydgoszcz, Bydgoszcz, Poland.

Background: Endothelial and platelet activation as well as a disruption of haemostatic balance are crucial in cancer-dependent venous thromboembolism development.

Objective: The aim of this study was to investigate the influence of von Willebrand factor (VWF), sE-selectin, sP-selectin as well as VWF/sE-selectin and sP-selectin/sE-selectin ratios on the probability of disease relapse in invasive breast carcinoma (IBrC) cases.

Methods: Eighty-four patients with IA-IIB stage of IBrC who passed a comprehensive clinicopathologic evaluation were included in the study. Read More

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http://dx.doi.org/10.3233/CBM-191096DOI Listing

New Therapeutic Targets and Treatment Options for Thrombotic Microangiopathy: Caplacizumab and Ravulizumab.

Authors:
Clement Chung

Ann Pharmacother 2020 07 25:1060028020941852. Epub 2020 Jul 25.

Houston Methodist West Hospital, Houston, TX, USA.

Objective: To review the efficacy and safety of caplacizumab and ravulizumab for thrombotic microangiopathy.

Data Sources: A literature search from January 2011 to May 2020 was performed using the key terms caplacizumab (or ALX-0681), ravulizumab (or ALXN1210), atypical hemolytic uremic syndrome (aHUS), acquired thrombotic thrombocytopenic purpura (aTTP), and thrombotic microangiopathy.

Study Selection And Data Extraction: Relevant clinical trials and articles in the English language were identified and reviewed. Read More

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http://dx.doi.org/10.1177/1060028020941852DOI Listing

Covid-19: The Rollercoaster of Fibrin(Ogen), D-Dimer, Von Willebrand Factor, P-Selectin and Their Interactions with Endothelial Cells, Platelets and Erythrocytes.

Int J Mol Sci 2020 Jul 21;21(14). Epub 2020 Jul 21.

Department of Physiological Sciences, Faculty of Science, Stellenbosch University, Stellenbosch 7602, South Africa.

Severe acute respiratory syndrome coronavirus 2 (SARS-Cov-2), also known as coronavirus disease 2019 (COVID-19)-induced infection, is strongly associated with various coagulopathies that may result in either bleeding and thrombocytopenia or hypercoagulation and thrombosis. Thrombotic and bleeding or thrombotic pathologies are significant accompaniments to acute respiratory syndrome and lung complications in COVID-19. Thrombotic events and bleeding often occur in subjects with weak constitutions, multiple risk factors and comorbidities. Read More

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http://dx.doi.org/10.3390/ijms21145168DOI Listing

Plasma Level of von Willebrand Factor Propeptide at Diagnosis: A Marker of Subsequent Renal Dysfunction in Autoimmune Rheumatic Diseases.

Clin Appl Thromb Hemost 2020 Jan-Dec;26:1076029620938874

Department of General Medicine, Nara Medical University, Kashihara, Nara, Japan.

Introduction: Patients with systemic autoimmune rheumatic diseases (SARDs) such as rheumatoid arthritis, systemic lupus erythematosus (SLE), Sjögren syndrome, and systemic sclerosis, which are chronic inflammatory diseases, are prone to develop renal dysfunction, which is related to vascular endothelial cell damage.

Material And Methods: We evaluated plasma levels of von Willebrand factor (VWF), VWF propeptide (VWF-pp), disintegrin-like and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), and VWF multimer pattern in patients with SARDs at diagnosis and investigated whether they may serve as markers to identify patients destined to develop renal dysfunction within 1 year. Renal dysfunction was defined as subsequent reduced estimated glomerular filtration rate (eGFR) by >25% or the new appearance of abnormal urine findings such as proteinuria (protein > 30 mg/dL) or hematuria (red blood cells >20/HPF in urine sediments). Read More

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http://dx.doi.org/10.1177/1076029620938874DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7383728PMC

Detection of acquired von Willebrand syndrome after ventricular assist device by total thrombus-formation analysis system.

ESC Heart Fail 2020 Jul 23. Epub 2020 Jul 23.

Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Center for Metabolic Regulation of Healthy Aging, Kumamoto University, Kumamoto, Japan.

Aims: Bleeding is a serious complication in patients with continuous-flow left ventricular assist device (CF-LVAD). Acquired von Willebrand syndrome (AVWS; type 2A) develops because of high shear stress inside the pumps and is a cause of bleeding complication. Although von Willebrand factor (vWF) multimer analysis is useful for diagnosing AVWS, it is only performed in specialized research institutes. Read More

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http://dx.doi.org/10.1002/ehf2.12824DOI Listing

A Novel Quantitative Method for Analyzing Desmopressin in Human Plasma using Liquid Chromatography-Tandem Mass Spectrometry.

Ther Drug Monit 2020 Jul 16. Epub 2020 Jul 16.

PharmD, PhD, Clinical Pharmacology and Hospital Pharmacy - Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Background: Desmopressin (D-amino D-arginine vasopressin: dDAVP) is used for the treatment of patients with hemophilia A and Von Willebrand disease. Studies on the rationale of dosing are scarce and mainly focus on the underlying causes of the vast differences in desmopressin response among individuals. The aim of this study was to develop and validate a liquid chromatography-tandem mass spectrometry (LC-MS/MS) method for the quantification of desmopressin in human plasma for identifying its pharmacokinetics and its therapeutic effect relationship in patients with bleeding disorder. Read More

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http://dx.doi.org/10.1097/FTD.0000000000000791DOI Listing

Von Willebrand factor (vWF): marker of endothelial damage and thrombotic risk in COVID-19?

Clin Med (Lond) 2020 Jul 21. Epub 2020 Jul 21.

Brighton and Sussex Medical School, Falmer, UK and consultant haematologist, Royal Sussex County Hospital, Brighton, UK

Background: COVID-19 infection is characterised, among other features, by a prothrombotic state with high rate of venous thromboembolism (VTE), D-dimer, and fibrinogen levels. Clinical observations have also highlighted that these patients have elevated von Willebrand factor (vWF) and factor VIIIc.

Methods: 24 consecutive COVID-19 positive patients were selected from the intensive care unit (ICU) or the high acuity ward of Brighton and Sussex University Hospitals NHS Trust. Read More

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http://dx.doi.org/10.7861/clinmed.2020-0346DOI Listing

Recombinant vs plasma-derived von Willebrand factor to prevent postpartum hemorrhage in von Willebrand disease.

Blood Adv 2020 Jul;4(14):3234-3238

Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh PA; Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh, Pittsburgh PA; and Hemophilia Center of Western Pennsylvania, Pittsburgh, PA.

von Willebrand disease (VWD) is a congenital bleeding disorder characterized by deficient or defective von Willebrand factor (VWF). Among women with VWD, postpartum hemorrhage (PPH) is common. Treatment options at delivery include plasma-derived VWF (pdVWF) and recombinant VWF (rVWF). Read More

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http://dx.doi.org/10.1182/bloodadvances.2020002046DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7391151PMC

Platelet disturbances correlate with endothelial cell activation in uncomplicated Plasmodium vivax malaria.

PLoS Negl Trop Dis 2020 Jul 20;14(7):e0007656. Epub 2020 Jul 20.

Laboratório de Doenças Tropicais-Prof. Luiz Jacintho da Silva. Instituto de Biologia, Universidade Estadual de Campinas, Campinas, Brazil.

Platelets drive endothelial cell activation in many diseases. However, if this occurs in Plasmodium vivax malaria is unclear. As platelets have been reported to be activated and to play a role in inflammatory response during malaria, we hypothesized that this would correlate with endothelial alterations during acute illness. Read More

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http://dx.doi.org/10.1371/journal.pntd.0007656DOI Listing

Determination of von Willebrand factor level in patient with sickle cell diseasein vaso-occlusive crisis.

Res Pract Thromb Haemost 2020 Jul 10;4(5):902-905. Epub 2020 Jul 10.

Department of Biochemistry Ebonyi State University Abakaliki Nigeria.

Background: Sickle cell anemia (SCA) is a hypercoagulable state characterized by a significant alteration in hemostatic parameters that may predispose to increased risk of vaso-occlusive crisis (VOC). The role of von Willebrand factor (VWF) in the pathogenesis of VOC has not been fully investigated in Nigeria.

Objective: The objective of this study was to evaluate the level of VWF in subjects with sickle cell disease (SCD) in Calabar, Nigeria, to determine its role in the pathogenesis of VOC. Read More

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http://dx.doi.org/10.1002/rth2.12378DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7354399PMC

Illustrated State-of-the-Art Capsules of the ISTH 2020 Congress.

Res Pract Thromb Haemost 2020 Jul 12;4(5):680-713. Epub 2020 Jul 12.

Department of Cell and Developmental Biology Perelman School of Medicine University of Pennsylvania Philadelphia PA USA.

The 2020 Congress of the International Society of Thrombosis and Haemostasis (ISTH) was held virtually July 12-15, 2019, due to the coronavirus disease 2019 pandemic. The congress convenes annually to discuss clinical and basic topics in hemostasis and thrombosis. Each year, the program includes State of Art (SOA) lectures given by prominent scientists. Read More

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http://dx.doi.org/10.1002/rth2.12368DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7354406PMC

Characterization of the von Willebrand factor/factor VIII complex produced by a novel purification process.

Arch Pharm Res 2020 Jul 14;43(7):714-723. Epub 2020 Jul 14.

R&D Center, GC Pharma, 93 Ihyeon-ro 30 Beon-Gil, Giheung-gu, Yongin, 16924, Republic of Korea.

Factor VIII (FVIII) is a blood coagulation protein that circulates as a complex with von Willebrand factor (vWF) in the plasma. In the survey of inhibitors in plasma product exposed toddlers (SIPPET) study, plasma-derived FVIII containing vWF was less immunogenic in hemophilia A patients than products with only high-purity FVIII only or recombinant FVIII. The  FVIII purified by the conventional purification process using anion-exchange (AEX) chromatography had a low vWF content. Read More

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http://dx.doi.org/10.1007/s12272-020-01245-yDOI Listing

Diagnostic challenges in von Willebrand disease. Report of two cases with emphasis on multimeric and molecular analysis.

Platelets 2020 Jul 14:1-4. Epub 2020 Jul 14.

Hemostasis and Eritropathology Unit, Hematopathology, Department of Pathology, CDB, Hospital Clínic, IDIBAPS, University of Barcelona , Barcelona, Spain.

Identification of qualitative variants of von Willebrand disease (VWD) can be a diagnostic challenge because of discrepant results obtained in the multiple laboratory tests available for its appropriate classification. We report two cases of infrequent inherited variants of VWD with unclear preliminary results with the test panel available at the time of first consultation and that were finally diagnosed as a VWD type 2A/IID with a c.8318 G > C, p. Read More

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http://dx.doi.org/10.1080/09537104.2020.1784403DOI Listing

Low VWF: insights into pathogenesis, diagnosis, and clinical management.

Blood Adv 2020 Jul;4(13):3191-3199

Irish Centre for Vascular Biology, School of Pharmacy and Biomolecular Sciences, Royal College of Surgeons in Ireland, Dublin, Ireland; National Coagulation Centre, St James's Hospital, Dublin, Ireland; and National Children's Research Centre, Our Lady's Children's Hospital Crumlin, Dublin, Ireland.

von Willebrand disease (VWD) constitutes the most common inherited human bleeding disorder. Partial quantitative von Willebrand factor (VWF) deficiency is responsible for the majority of VWD cases. International guidelines recommend that patients with mild to moderate reductions in plasma VWF antigen (VWF:Ag) levels (typically in the range of 30-50 IU/dL) should be diagnosed with low VWF. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020002038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7362371PMC

Non-invasive risk stratification after HCV-eradication in patients with advanced chronic liver disease.

Hepatology 2020 Jul 13. Epub 2020 Jul 13.

Division of Gastroenterology and Hepatology, Department of Internal Medicine III, Medical University of Vienna, Vienna, Austria.

Background & Aims: Risk stratification after cure from hepatitis C virus (HCV) infection remains a clinical challenge. We investigated the predictive value of non-invasive surrogates of portal hypertension (liver stiffness measurement (LSM) by vibration-controlled transient elastography and von Willebrand factor/platelet count ratio (VITRO)) for development of hepatic decompensation and hepatocellular carcinoma in patients with pre-treatment advanced chronic liver disease (ACLD) who achieved HCV-cure.

Approach & Results: 276 patients with pre-treatment ACLD and information on pre- and post-treatment (follow-up (FU))-LSM and -VITRO were followed for a median of 36. Read More

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http://dx.doi.org/10.1002/hep.31462DOI Listing

Endothelial characteristics in healthy endothelial colony forming cells; generating a robust and valid ex vivo model for vascular disease.

J Thromb Haemost 2020 Jul 12. Epub 2020 Jul 12.

Department of Internal medicine, division of Thrombosis and Hemostasis, Einthoven Laboratory for Vascular and Regenerative Medicine, Leiden University Medical Center, Leiden, the Netherlands.

Background: Endothelial colony forming cells (ECFCs) derived from peripheral blood can be used to analyze the pathophysiology of vascular diseases ex vivo. However, heterogeneity is observed between ECFC clones and this variability needs to be understood and standardized for ECFCs to be used as a cell model for applications in vascular studies.

Objective: Determine reference characteristics of healthy control ECFCs to generate a valid ex vivo model for vascular disease. Read More

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http://dx.doi.org/10.1111/jth.14998DOI Listing

Pulsatility in ventricular assistance devices: A translational review focused on applied haemodynamics.

Arch Cardiovasc Dis 2020 Jun - Jul;113(6-7):461-472. Epub 2020 Jul 9.

Department of Cardiac Surgery, Pitié-Salpêtrière Hospital, AP-HP, Sorbonne University, 75013 Paris, France; Department of Research and Innovation (RICAP), CMC Ambroise Paré, 92200 Neuilly-sur-Seine, France.

Heart failure affects more than 30 million people worldwide and its prevalence is constantly rising. In 2020, heart transplantation is the only curative treatment, but left ventricular assistance devices (LVADs) are fully integrated into the decision algorithm for management of patients with advanced heart failure, with more than 20,000 devices implanted worldwide in the last decade. Intended to support cardiac output, LVADs remove the blood from the left ventricle and eject it into the proximal aorta. Read More

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http://dx.doi.org/10.1016/j.acvd.2020.03.017DOI Listing

Decreased RPM reduces von Willebrand factor degradation with the EVAHEART LVAS: implications for device-specific LVAD management.

J Card Surg 2020 Jul;35(7):1477-1483

Evaheart Inc, Houston, Texas.

Background: Continuous-flow left ventricular assist devices (LVADs) produces supraphysiologic shear stress that causes von Willebrand factor (VWF) degradation and a bleeding diathesis. Reduction of revolutions per minute (RPM) with axial-flow LVADs does not decrease shear stress enough to reduce VWF degradation and bleeding. However, it is unknown if RPM reduction with centrifugal flow LVADs may minimize VWF degradation. Read More

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http://dx.doi.org/10.1111/jocs.14620DOI Listing

Acquired von Willebrand syndrome secondary to mitral and aortic regurgitation.

J Card Surg 2020 Jul 11. Epub 2020 Jul 11.

Department of Cardiovascular Surgery, National Hospital Organization Tokyo Medical Center, Meguro, Tokyo, Japan.

Background: The association of aortic valve stenosis with gastrointestinal bleeding was first described by Edward Heyde in 1958. Since then, there have been numerous case reports of Heyde syndrome in the medical literature worldwide.

Aims: Recently, the definition of Heyde syndrome has been updated to include the combination of aortic valve stenosis, intestinal angiodysplasia, and acquired von Willebrand factor syndrome (AVWS). Read More

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http://dx.doi.org/10.1111/jocs.14796DOI Listing

Apixaban Downregulates Endothelial Inflammatory and Prothrombotic Phenotype in an In Vitro Model of Endothelial Dysfunction in Uremia.

Cardiovasc Drugs Ther 2020 Jul 10. Epub 2020 Jul 10.

Pathology Department, CDB, Hospital Clínic, IDIBAPS, University of Barcelona, Barcelona, Spain.

Purpose: Chronic kidney disease (CKD) associates with inflammatory and prothrombotic phenotypes, resulting in higher cardiovascular risk. Factor Xa displays functions beyond coagulation, exhibiting proinflammatory effects. The aim of the present study was to investigate whether a direct FXa inhibitor protects from the endothelial dysfunction (ED) caused by uremia. Read More

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http://dx.doi.org/10.1007/s10557-020-07010-zDOI Listing

Acquired von Willebrand Syndrome Hiding Inherited von Willebrand Disease Can Explain Severe Bleeding in Patients With Aortic Stenosis.

Arterioscler Thromb Vasc Biol 2020 Jul 9:ATVBAHA120314656. Epub 2020 Jul 9.

From the Department of Medicine, First Chair of Internal Medicine, University of Padua Medical School, Italy. (A.C., E.G., G.B., V.D.).

Objective: Aortic stenosis may be complicated by an acquired von Willebrand syndrome that rarely causes significant bleeding, raising the question of why it does so in a few cases. To seek an explanation, we studied 5 severe bleeder aortic stenosis patients in a cohort of 49 patients, using the flowchart for inherited von Willebrand disease. Approach and Results: All 5 patients were lacking in large and intermediate VWF (von Willebrand factor) multimers, 3 had reduced plasma and platelet VWF levels, and none showed PFA100 closure. Read More

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http://dx.doi.org/10.1161/ATVBAHA.120.314656DOI Listing

A microchip flow-chamber assay screens congenital primary hemostasis disorders.

Pediatr Int 2020 Jul 8. Epub 2020 Jul 8.

Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan.

Background: Von Willebrand disease (VWD) and platelet function disorders (PFDs) are congenital bleeding disorders caused by primary hemostasis defects. Platelet function tests are time-consuming and require considerable amounts of blood sample, and there were no easy-to-use assays for assessing platelet function quickly and sensitively. We reported the usefulness of a microchip flow-chamber system (T-TAS ) for detecting and/or predicting clinical severities in patients with VWD type 1 and 2N and platelet storage pool disease. Read More

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http://dx.doi.org/10.1111/ped.14378DOI Listing

Association of Markers of Microvascular Dysfunction With Prevalent and Incident Depressive Symptoms: The Maastricht Study.

Hypertension 2020 Aug 8;76(2):342-349. Epub 2020 Jul 8.

Department of Internal Medicine (A.F.J.G., M.J.M.v.A., C.D.A.S., B.M.S., N.C.S., R.M.A.H., C.J.H.v.d.K., T.T.v.S., M.T.S., A.J.H.M.H.), Maastricht University Medical Center (MUMC+), the Netherlands.

The etiology of late-life depression (LLD) is still poorly understood. Microvascular dysfunction (MVD) has been suggested to play a role in the etiology of LLD, but direct evidence of this association is scarce. The aim of this study was to investigate whether direct and indirect markers of early microvascular dysfunction are associated with prevalent and incident LLD in the population-based Maastricht Study cohort. Read More

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http://dx.doi.org/10.1161/HYPERTENSIONAHA.120.15260DOI Listing

ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP.

Blood Adv 2020 Jul;4(13):3093-3101

Department of Nephrology and Hypertension, Medical School Hannover, Hannover, Germany.

Introduction of the nanobody caplacizumab was shown to be effective in the treatment of acquired thrombotic thrombocytopenic purpura (aTTP) in the acute setting. The official recommendations include plasma exchange (PEX), immunosuppression, and the use of caplacizumab for a minimum of 30 days after stopping daily PEX. This study was a retrospective, observational analysis of the use of caplacizumab in 60 patients from 29 medical centers in Germany. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020001987DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7362349PMC

The Effect of Age on von Willebrand Factor and Bleeding Symptoms in von Willebrand Disease.

Thromb Haemost 2020 Aug 6;120(8):1159-1165. Epub 2020 Jul 6.

Division of Hematology/Oncology, Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, United States.

von Willebrand disease (VWD) is a quantitative or qualitative defect in von Willebrand factor (VWF) resulting in mucocutaneous bleeding symptoms and hemorrhage following hemostatic challenges, such as trauma or surgery. VWD-specific therapy, DDAVP (1-desamino-8-D-arginine vasopressin) and VWF concentrates, is necessary periprocedurally to ensure adequate hemostasis. The aging VWD patient may complicate this matter. Read More

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http://dx.doi.org/10.1055/s-0040-1713636DOI Listing

Thrombotic microangiopathy, DIC-syndrome and COVID-19: link with pregnancy prothrombotic state.

J Matern Fetal Neonatal Med 2020 Jul 6:1-9. Epub 2020 Jul 6.

Obstetrics and Gynecology, The First I.M. Sechenov Moscow State Medical University, (Sechenov University), Moscow, Russia.

For last months, humanity has faced a formidable unknown enemy, which is presented as a new coronavirus infection. Despite the fact that the causative agents of new diseases appear at a certain frequency and that the virus SARS-CoV-2 has certain common properties with its predecessors, at the moment we are dealing with a new unknown pathogenesis of the development of severe complications in patients with risk factors. A final understanding of pathological process mechanisms is the goal of the scientific community. Read More

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http://dx.doi.org/10.1080/14767058.2020.1786811DOI Listing

Development of a receptor-based inhibitory penta-unit-conjugated peptide to enhance anthrax toxin neutralization.

Int J Biol Macromol 2020 Jul 1;163:327-335. Epub 2020 Jul 1.

Department of Chemistry and Research Institute for Natural Sciences, Hanyang University, Seoul 04763, Republic of Korea. Electronic address:

Anthrax toxin is a key virulence factor for Bacillus anthracis. The cell-binding component of anthrax toxin, protective antigen (PA), mediates the entry of the toxin into cells by first binding to the extracellular von Willebrand factor A (VWA) domain of the cellular anthrax toxin receptor (ATR). Herein, we targeted the VWA domain of the cellular receptor to develop a more effective antitoxin agent for neutralization of anthrax toxin. Read More

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http://dx.doi.org/10.1016/j.ijbiomac.2020.06.264DOI Listing
July 2020
2.858 Impact Factor

Endotheliopathy in COVID-19-associated coagulopathy: evidence from a single-centre, cross-sectional study.

Lancet Haematol 2020 Aug 30;7(8):e575-e582. Epub 2020 Jun 30.

Section of Hematology, New Haven, CT, USA. Electronic address:

Background: An important feature of severe acute respiratory syndrome coronavirus 2 pathogenesis is COVID-19-associated coagulopathy, characterised by increased thrombotic and microvascular complications. Previous studies have suggested a role for endothelial cell injury in COVID-19-associated coagulopathy. To determine whether endotheliopathy is involved in COVID-19-associated coagulopathy pathogenesis, we assessed markers of endothelial cell and platelet activation in critically and non-critically ill patients admitted to the hospital with COVID-19. Read More

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http://dx.doi.org/10.1016/S2352-3026(20)30216-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7326446PMC

Endothelial Activation, Acute Kidney Injury, and Cognitive Impairment in Pediatric Severe Malaria.

Crit Care Med 2020 Jun 30. Epub 2020 Jun 30.

1Department of Medical Microbiology, College of Health Sciences, Makerere University, Kampala, Uganda. 2Department of Epidemiology and Biostatistics, College of Health Sciences, Makerere University, Kampala, Uganda. 3Department of Pediatrics, Ryan White Center for Pediatric Infectious Disease and Global Health, Indiana University School of Medicine, Indianapolis, IN. 4Department of Pediatrics, University of Minnesota, Minneapolis, MN. 5Department of Paediatrics and Child Health, Makerere University College of Health Sciences, Kampala, Uganda. 6Nuffield Department of Medicine, Centre of Tropical Medicine and Global Health, University of Oxford, Oxford, United Kingdom. 7Department of Psychiatry, College of Health Sciences, Makerere University, Kampala, Uganda. 8Department of Medicine, University of Toronto and University Health Network, Toronto, ON, Canada.

Objectives: Evaluate the relationship between endothelial activation, malaria complications, and long-term cognitive outcomes in severe malaria survivors.

Design: Prospectively cohort study of children with cerebral malaria, severe malarial anemia, or community children.

Setting: Mulago National Referral Hospital in Kampala, Uganda. Read More

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http://dx.doi.org/10.1097/CCM.0000000000004469DOI Listing

Case Report: an İnfant with Severe Thrombocytopenia Diagnosed with Type 2B von Willebrand Disease Due to a de novo p.Val1316Met Mutation.

Turk J Haematol 2020 Jul 3. Epub 2020 Jul 3.

Department of hematology and oncology, Children's Hospital of Soochow University.

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http://dx.doi.org/10.4274/tjh.galenos.2020.2020.0213DOI Listing

Novel antiplatelet strategies targeting VWF and GPIb.

Platelets 2020 Jul 2:1-5. Epub 2020 Jul 2.

Department of Haematology, University College London Hospital , London, UK.

Von Willebrand factor has a pivotal role in primary hemostasis. Its role in thrombotic microangiopathies (TMA), as well as cardiovascular disease, has been demonstrated. Thrombotic thrombocytopenic purpura (TTP), a thrombotic microangiopathy, is a life-threatening condition with a high mortality rate if untreated. Read More

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http://dx.doi.org/10.1080/09537104.2020.1786038DOI Listing