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acquired von willebrand

I Mancini, I Ricaño-Ponce, E Pappalardo, A Cairo, M M Gorski, G Casoli, B Ferrari, M Alberti, D Mikovic, M Noris, C Wijmenga, F Peyvandi
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy associated with the development of autoantibodies against the von Willebrand factor cleaving protease, ADAMTS13. Similarly to other autoimmune disorders, evidences of a genetic contribution have been reported, including the association of the human leukocyte antigen (HLA) class II complex with disease risk OBJECTIVE: To identify novel genetic risk factors in acquired TTP...
October 20, 2016: Journal of Thrombosis and Haemostasis: JTH
Jonathan S Harrison, Shellaine R Frazier, Dianne D McConnell, Senan John Yasar, Nataliya Melnyk, Gratian Salaru
Acquired coagulopathies are common; uncommonly, adsorption of coagulation factors from the circulation into the tissues by pathologic amyloid exceeds the body's ability to produce factor and results in acquired factor deficiency. When amyloidosis does cause a coagulopathy, it is most often acquired factor X deficiency, but there are rare reports of amyloidosis being associated with other acquired factor deficiencies. We investigated a case of a severe bleeding diathesis, the cause of which was combined acquired factor V deficiency and concomitant acquired von Willebrand syndrome...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
M Martinuzzo, L Barrera, D Altuna, F Tisi Baña, J Bieti, Q Amigo, M D'Adamo, M S López, J Oyhamburu, J C Otaso
BACKGROUND: Homozygous or double heterozygous factor XIII (FXIII) deficiency is characterized by soft tissue hematomas, intracranial and delayed spontaneous bleeding. Alterations of thromboelastography (TEG) parameters in these patients have been reported. The aim of the study was to show results of TEG, TEG Lysis (Lys 60) induced by subthreshold concentrations of streptokinase (SK), and to compare them to the clot solubility studies results in samples of a 1-year-old girl with homozygous or double heterozygous FXIII deficiency...
2016: Mediterranean Journal of Hematology and Infectious Diseases
Emmanuel J Favaloro
von Willebrand Disease (VWD) is the most common inherited bleeding disorder and also arises as an acquired defect (AVWS). VWD and AVWS are due to quantitative deficiencies and/or qualitative defects in von Willebrand factor (VWF), an adhesive plasma protein with multiple activities. Diagnosis of VWD is problematic, being subject to overdiagnosis, underdiagnosis, and misdiagnosis. This is largely due to limitations in current test procedures and an over-reliance on these imperfect test systems for clinical diagnosis...
September 13, 2016: American Journal of Hematology
Emma Fosbury, Elwira Szychot, Olga Slater, Mary Mathias, Keith Sibson
Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology...
September 12, 2016: Pediatric Blood & Cancer
P Lukito, A Wong, J Jing, J F Arthur, S F Marasco, D A Murphy, P J Bergin, J A Shaw, M Collecutt, R K Andrews, E E Gardiner, A K Davis
BACKGROUND: Ventricular assist devices (VADs) and extracorporeal membrane oxygenation (ECMO) are associated with bleeding, not fully explained by anti-coagulant or anti-platelet use. Exposure of platelets to elevated shear in vitro leads to increased shedding. OBJECTIVES: Here we investigated whether loss of platelet receptors occurs in vivo, and the relationship with acquired Von Willebrand Syndrome (AVWS) METHODS: Platelet counts, coagulation tests and Von Willebrand factor (VWF) analyses were performed on samples from 21 continuous flow VAD (CF-VAD), 20 ECMO, 12 heart failure and 7 aortic stenosis patients...
September 7, 2016: Journal of Thrombosis and Haemostasis: JTH
Nathan Gossai, Nicholas M Brown, Rebecca Ameduri, Nicole D Zantek, James St Louis, Marie E Steiner
BACKGROUND: The balance of hemostasis and anticoagulation is a concern for patients dependent upon ventricular assist devices (VADs). Bleeding is a common complication with both short- and long-term use of these devices. A better understanding of the risk factors and etiologies of bleeding associated with these devices is needed and could improve the overall results. We sought to determine the relationship of mechanical circulatory assist device use with acquired von Willebrand disease (avWD) in children...
September 2016: World Journal for Pediatric & Congenital Heart Surgery
H Y Ouyang, Z J Yu, J Yin, X J Zhao, Z Y Wang, W Zhang, Z N Ma, J Su, X Bai, C G Ruan
OBJECTIVE: To deepen the understanding of acquired von Willebrand syndrome (AVWS). METHODS: The clinical data of 3 patients were analyzed and related literature were reviewed. RESULTS: ① Case 1, a 70- year- old male, diagnosed as Waldenstrom macroglobulinemia and AVWS, was presented with spontaneous epitaxis and bruising. The VWF∶Ag level was 16%. Treatment was initiated with VWF concentrates. Two cycles of chemotherapy with Bortezomib, thalidomide and Dexamethasone were followed...
August 14, 2016: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
Qasam M Ghulam, Kim K Bredahl, Jørgen B Gram, Lars Lönn, Jens P Goetze, Henrik H Sillesen, Jonas P Eiberg
OBJECTIVES: Disruption of the endothelial lining may be one of the events linking intraluminal thrombus and abdominal aortic aneurysm growth. In the present study, we examined whether von Willebrand factor activity in plasma, contact proteins of blood coagulation, and inflammatory biomarkers may be associated with intraluminal thrombus volume in search of a biochemical marker of endothelial damage and thrombus size. DESIGN: Prospective study, correlating potential endothelial biomarkers and intraluminal thrombus volume acquired by computed tomography angiography...
August 2016: Vascular and Endovascular Surgery
Rowena C Punzalan, Jerome L Gottschall
Although congenital bleeding disorders can manifest in the newborn period, the most common causes of bleeding and thrombosis in neonates are acquired conditions. Factor concentrates are used for specific diagnoses (hemophilia with inhibitors, specific factor deficiency, von Willebrand disease) and approved indications, and increasingly for off-label indications (bleeding in surgery cardiopulmonary bypass, extracorporeal membrane oxygenation). We will review the approved indications for factor products in the neonate and discuss the evidence and rationale for off-label use of factor products in management of bleeding and thrombosis in the neonate...
October 2016: Transfusion Medicine Reviews
Ian B Hollis, Sheh-Li Chen, Patricia P Chang, Jason N Katz
Patients with a durable, continuous-flow left ventricular assist device (LVAD) are commonly prescribed the combination of an oral anticoagulant and an oral antiplatelet agent as prophylaxis against device thrombosis and systemic embolic events. Current guidelines recommend warfarin with an INR goal of 2-3 and concomitant aspirin 81-325mg daily for patients with a HeartMate II ® LVAD (HM II). Unfortunately, gastrointestinal bleeding (GIB) is very common in these patients due to multiple factors including the development of arteriovenous malformations (AVMs) and acquired von Willebrand syndrome (AvWS)...
August 22, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Mari Tinholt, Per Morten Sandset, Marie-Christine Mowinckel, Øystein Garred, Kristine Kleivi Sahlberg, Vessela N Kristensen, Anne-Lise Børresen-Dale, Anne Flem Jacobsen, Grethe Skretting, Nina Iversen
BACKGROUND: We have previously reported acquired activated protein C (APC) resistance and elevated plasma D-dimer levels in breast cancer patients. Here, we aimed to identify phenotypic and genetic determinants that contribute to the acquired APC resistance and increased D-dimer levels in breast cancer. Healthy controls served as reference. We also addressed whether higher APC resistance or D-dimer levels could be potential markers of clinicopathological breast cancer characteristics...
September 2016: Thrombosis Research
Fatemeh Esmaeilzadeh, Aurélien Wauters, Walter Wijns, Jean-François Argacha, Philippe van de Borne
BACKGROUND: Left Ventricular Assist Device (LVAD) is a promising therapy for patients with advanced heart failure (HF), but bleeding complications remain an important issue. Previous series show that acquired von Willebrand syndrome was present in up to 100 % of first generation LVAD recipients. We report the effects of new generation LVADs on vW factor (vWF) metabolism and activity in our center. METHODS: Fifteen LVAD recipients (HeartWare®, Framingham, MA, USA) were compared to 12 HF patients, matched for age and body mass index...
2016: BMC Cardiovascular Disorders
Baohong Zou, Xuexue Hong, Yuan Ding, Xiang Wang, He Liu, Jian Hua
Copines are evolutionarily conserved calcium-dependent membrane-binding proteins with potentially critical biological functions. In plants, the function of these proteins has not been analyzed except for in Arabidopsis thaliana where they play critical roles in development and disease resistance. To facilitate functional studies of copine proteins in crop plants, genome-wide identification, curation, and phylogeny analysis of copines in 16 selected plant species were conducted. All the identified 32 plant copines have conserved features of the two C2 domains (C2A and C2B) and the von Willebrand factor A (vWA) domain...
August 2016: Genome Génome / Conseil National de Recherches Canada
Rolf Dario Frank, Regina Lanzmich, Philipp K Haager, Ulrich Budde
Aortic valve stenosis (AVS) is the most common valve disease in adults. Severe forms are associated with acquired von Willebrand syndrome (aVWS) with loss of the largest von Willebrand factor (VWF) multimers. Diagnostic gold standard is the VWF multimer analysis. Valve replacement rapidly restores the VWF structure. Uncertainty exists if this effect is permanent and how functional VWF assays perform compared with multimer analysis. We studied 21 consecutive patients with severe AVS before and 6 to 18 months after valve surgery and compared them with 14 controls without valve disease referred for coronary angiography...
August 1, 2016: Clinical and Applied Thrombosis/hemostasis
Louis Deforche, Claudia Tersteeg, Elien Roose, Aline Vandenbulcke, Nele Vandeputte, Inge Pareyn, Elien De Cock, Hanspeter Rottensteiner, Hans Deckmyn, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy linked to a deficiency in the metalloprotease ADAMTS13. In the current study, a novel mouse model for acquired TTP was generated to facilitate development and validation of new therapies for this disease. Therefore, a large panel (n = 19) of novel anti-mouse ADAMTS13 (mADAMTS13) monoclonal antibodies (mAbs) of mouse origin was generated. Inhibitory anti-mADAMTS13 mAbs were identified using the FRETS-VWF73 assay. Four mAbs strongly inhibited mADAMTS13 activity in vitro (∼68-90% inhibition)...
2016: PloS One
Yoshikazu Nagase, Shuji Ueda, Hitomi Matsunaga, Aya Yoshioka, Yoshiyuki Okada, Tomohisa Machida, Keiichi Nakata, Fuka Mima, Risato Takeda, Daisuke Hayashi, Sadaharu Iio, Kohei Okita, Hiroyuki Narahara, Yuichi Yasunaga, Yoshiaki Inui, Sumio Kawata
BACKGROUND: Acute compartment syndrome is an orthopedic emergency requiring urgent fasciotomy to prevent irreversible damage. In hematological malignancies, acute compartment syndrome caused by severe soft tissue bleeding is extremely rare. We present a patient with chronic-phase chronic myeloid leukemia who had acute compartment syndrome caused by severe soft tissue bleeding in her right forearm. CASE PRESENTATION: A 72-year-old Japanese woman was referred to our hospital with swelling and pain of her right forearm without a previous history of trauma...
2016: Journal of Medical Case Reports
William B Hillegass, Nita A Limdi
No abstract text is available yet for this article.
May 1, 2016: JAMA Cardiology
Joseph L Blackshear, Chad W McRee, Robert E Safford, Peter M Pollak, Mark E Stark, Colleen S Thomas, Candido E Rivera, Ewa M Wysokinska, Dong Chen
IMPORTANCE: Limited data suggest that von Willebrand factor (VWF) abnormalities may accompany the high-shear state associated with prosthetic valve dysfunction. If true, laboratory testing could add value in quantifying prosthesis dysfunction and could suggest a pathophysiological explanation for acquired bleeding in some patients. OBJECTIVES: To determine whether dysfunctional valve prostheses are associated with VWF abnormalities compared with normally functioning valve prostheses, to identify the severity of the VWF abnormality relative to other conditions, and to describe associated bleeding and the occurrence of gastrointestinal angiodysplasia...
May 1, 2016: JAMA Cardiology
John Chapin, Jaqueline Bamme, Fraustina Hsu, Paul Christos, Maria DeSancho
Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed...
July 14, 2016: Clinical and Applied Thrombosis/hemostasis
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