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https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#1
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28382967/thrombotic-thrombocytopenic-purpura
#2
REVIEW
Johanna A Kremer Hovinga, Paul Coppo, Bernhard Lämmle, Joel L Moake, Toshiyuki Miyata, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP; also known as Moschcowitz disease) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic haemolytic anaemia and a variable degree of ischaemic organ damage, particularly affecting the brain, heart and kidneys. Acute TTP was almost universally fatal until the introduction of plasma therapy, which improved survival from <10% to 80-90%. However, patients who survive an acute episode are at high risk of relapse and of long-term morbidity...
April 6, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28367342/remission-of-severe-relapsed-and-refractory-ttp-after-multiple-cycles-of-bortezomib
#3
Manu R Pandey, Pankit Vachhani, Evelena P Ontiveros
Acquired thrombotic thrombocytopenic purpura (TTP) is characterized by autoantibodies against a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13). Uncleaved von Willebrand factor (VWF) multimers accumulate and bind to platelets which causes spontaneous microthrombi ultimately causing microangiopathic hemolytic anemia, thrombocytopenia, and end-organ ischemia. Plasma exchange (PEX) with or without steroids constitutes standard first-line therapy with rituximab typically reserved for refractory cases...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28319521/warfarin-and-aspirin-versus-warfarin-alone-for-prevention-of-embolic-events-in-patients-with-a-heartmate-ii%C3%A2-left-ventricular-assist-device
#4
Joseph S Van Tuyl, Ian B Hollis, Khalid A Alburikan, Richard Tran, Brian P Murray, Jo E Rodgers, Jason N Katz, Brett C Sheridan
Acquired von Willebrand disease increases bleeding risk in patients implanted with a continuous-flow left ventricular assist device. Lower aspirin doses decrease the risk of bleeding without an increased risk of embolic events. No published studies in the U.S. have compared the incidence of bleeding and thrombotic events between antithrombotic regimens with and without aspirin. A single-center, retrospective analysis was conducted of adult patients implanted with a HM II. Patients received warfarin and aspirin 81 mg daily or warfarin alone...
March 17, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28296884/increased-von-willebrand-factor-decreased-adamts13-and-thrombocytopenia-in-melioidosis
#5
Emma Birnie, Gavin C K W Koh, Ester C Löwenberg, Joost C M Meijers, Rapeephan R Maude, Nicholas P J Day, Sharon J Peacock, Tom van der Poll, W Joost Wiersinga
BACKGROUND: Melioidosis, caused by bioterror treat agent Burkholderia pseudomallei, is an important cause of community-acquired Gram-negative sepsis in Southeast Asia and Northern Australia. New insights into the pathogenesis of melioidosis may help improve treatment and decrease mortality rates from this dreadful disease. We hypothesized that changes in Von Willebrand factor (VWF) function should occur in melioidosis, based on the presence of endothelial stimulation by endotoxin, pro-inflammatory cytokines and thrombin in melioidosis, and investigated whether this impacted on outcome...
March 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28250305/splenic-marginal-zone-lymphoma-with-acquired-von-willebrand-syndrome-diagnosed-via-splenic-bleeding
#6
Yukiko Komeno, Naoki Shibuya, Hideki Uryu, Haruki Yamada, Takeo Toda, Masayuki Shibasaki, Shinji Kunishima, Kuniko Iihara, Tomiko Ryu
An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28236847/is-von-willebrand-disease-linked-to-cholesteatoma-aetiology
#7
 Reis Rego, M Santos, M Coutinho, T Feliciano, C Almeida E Sousa
Cholesteatoma is a common clinical picture seen by otolaryngologists. The disease is characterised by an abnormal grow "of skin in the wrong place". Specifically, in acquired cholesteatoma, the main causative issue is associated with ventilatory deficits within the middle ear and results in chronic infection. Molecular science has associated the presence of specific molecules with its development, and moreover recent research suggests that deregulated angiogenesis is a crucial process in the development of cholesteatoma and its recurrence...
March 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/28187047/is-extracorporeal-co2-removal-really-safe-and-less-invasive-observation-of-blood-injury-and-coagulation-impairment-during-ecco2r
#8
Johannes Kalbhenn, Nadine Neuffer, Barbara Zieger, Axel Schmutz
Extracorporeal CO2-Removal (ECCO2R) is promoted with attributes like "safe" and "less invasive" compared to (high-flow) veno-venous ECMO-Systems. With our experience in coagulation disorders during ECMO-therapy with this observational study we for the first time prospectively evaluate hemolysis and coagulation disorders during ECCO2R. Eight consecutive patients with predominant hypercapnic respiratory failure were treated with the Hemolung® Respiratory Assist System (RAS) (Alung-Technologies, Pittsburg, USA)...
February 7, 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28181217/hypercoagulability-and-migraine
#9
Gretchen E Tietjen, Stuart A Collins
BACKGROUND: A growing body of literature suggests that migraineurs, particularly those with aura, have an increased risk for ischemic stroke, but not via enhanced atherosclerosis. The theory that micro-emboli induced ischemia provokes cortical spreading depression (ie, symptomatic aura) in migraineurs but transient ischemic attacks in others highlights a potential role for hypercoagulability as a link between migraine (with aura) and stroke. AIM: Our objective is to summarize the literature evaluating the association of migraine with various acquired or inheritable thrombophilic states, including those related to elevated estrogen levels, endothelial activation and dysfunction, antiphospholipid antibodies (aPL), deficiency of coagulation inhibitors, and presence of certain genetic polymorphisms...
February 9, 2017: Headache
https://www.readbyqxmd.com/read/28110841/treatment-of-autoimmune-thrombotic-thrombocytopenic-purpura-in-the-more-severe-forms
#10
REVIEW
Paul Coppo
Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of >80%. However, relapses occur in up to 40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates...
February 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28103720/crohn-s-disease-and-acquired-von-willebrand-syndrome-a-rare-dangerous-affair
#11
Federico Pasin, Sophie Testa, Pietro Capone, Federico Buffoli, Antonio Cuzzoli, Giovanni Paolo Coppeta, Roberto Grassia
No abstract text is available yet for this article.
January 20, 2017: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/28088607/transcatheter-aortic-valve-implantation-leads-to-a-restoration-of-von-willebrand-factor-vwf-abnormalities-in-patients-with-severe-aortic-stenosis-incidence-and-relevance-of-clinical-and-subclinical-vwf-dysfunction-in-patients-undergoing-transfemoral-tavi
#12
Alexander Sedaghat, Hannah Kulka, Jan-Malte Sinning, Nora Falkenberg, Julia Driesen, Barbara Preisler, Christoph Hammerstingl, Georg Nickenig, Bernd Pötzsch, Johannes Oldenburg, Hans-Jörg Hertfelder, Nikos Werner
BACKGROUND: In this study, we sought to analyze the incidence and relevance of von Willebrand factor (VWF) abnormalities in patients undergoing transcatheter aortic valve implantation (TAVI), especially on perioperative bleeding. Furthermore, we hypothesized that, similar to aortic valve surgery, TAVI results in a restoration of VWF abnormalities. METHODS AND RESULTS: We performed a prospective analysis of periinterventional VWF parameters in 74 patients (80±7years, female in 37...
January 7, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28060126/pediatric-acquired-von-willebrand-syndrome-in-cardiopulmonary-disorders-do-laboratory-abnormalities-predict-bleeding-risk
#13
Saman K Hashmi, Mireya P Velasquez, Donald L Yee, Shiu-Ki Hui, Donald Mahoney, Lakshmi V Srivaths
There are conflicting reports on whether or not laboratory abnormalities in pediatric acquired von Willebrand syndrome (AVWS) predict bleeding manifestations in patients with cardiopulmonary disorders (CPD). We retrospectively reviewed charts of patients with AVWS and CPD (n=16) seen at Texas Children's Hospital from 2003 to 2012. The most common CPD were valve stenoses, ventricular septal defects, and pulmonary hypertension. All patients had loss of high molecular weight multimers. Fifteen (94%) patients presented with bleeding symptoms, with menorrhagia and epistaxis being the most common...
March 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28035064/functional-assessment-of-von-willebrand-factor-expression-by-cancer-cells-of-non-endothelial-origin
#14
Anahita Mojiri, Konstantin Stoletov, Maria Areli Lorenzana Carrillo, Lian Willetts, Saket Jain, Roseline Godbout, Paul Jurasz, Consolato M Sergi, David D Eisenstat, John D Lewis, Nadia Jahroudi
Von Willebrand factor (VWF) is a highly adhesive procoagulant molecule that mediates platelet adhesion to endothelial and subendothelial surfaces. Normally it is expressed exclusively in endothelial cells (ECs) and megakaryocytes. However, a few studies have reported VWF detection in cancer cells of non-endothelial origin, including osteosarcoma. A role for VWF in cancer metastasis has long been postulated but evidence supporting both pro- and anti-metastatic roles for VWF has been presented. We hypothesized that the role of VWF in cancer metastasis is influenced by its cellular origin and that cancer cell acquisition of VWF expression may contribute to enhanced metastatic potential...
February 21, 2017: Oncotarget
https://www.readbyqxmd.com/read/28028990/acquired-von-willebrand-syndrome
#15
REVIEW
Andrzej Mital
Acquired von Willebrand syndrome is a rare hemorrhagic diathesis, with clinical symptoms similar to those associated with the inherited form von Willebrand disease. This syndrome is characterized by a lack of previous bleeding symptoms, negative familial history, and occurrence in a relatively older age. Most commonly, acquired von Willebrand syndrome develops in the course of other conditions, such as lymphoproliferative, myeloproliferative, cardiovascular and autoimmune disorders; additionally, it can be associated with some non-hematological malignancies and use of certain prescription drugs...
November 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28017358/differentiation-of-mesenchymal-stem-cells-from-human-amniotic-fluid-to-vascular-endothelial-cells
#16
Waleephan Tancharoen, Sirinda Aungsuchawan, Peraphan Pothacharoen, Runchana Markmee, Suteera Narakornsak, Junjira Kieodee, Nonglak Boonma, Witoon Tasuya
Endothelial dysfunction is a principle feature of vascular-related disease. Endothelial cells have been acquired for the purposes of the restoration of damaged tissue in therapeutic angiogenesis. However, their use is limited by expansion capacity and the small amount of cells that are obtained. Human amniotic fluid mesenchymal stem cells (hAF-MSCs) are considered an important source for vascular tissue engineering. In this study, hAF-MSCs were characterized and then induced in order to differentiate into the endothelial-like cells...
March 2017: Acta Histochemica
https://www.readbyqxmd.com/read/28011677/n-acetylcysteine-in-preclinical-mouse-and-baboon-models-of-thrombotic-thrombocytopenic-purpura
#17
Claudia Tersteeg, Jan Roodt, Walter J Van Rensburg, Charlotte Dekimpe, Nele Vandeputte, Inge Pareyn, Aline Vandenbulcke, Barbara Plaimauer, Seb Lamprecht, Hans Deckmyn, José A Lopez, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder diagnosed by thrombocytopenia and hemolytic anemia, associated with a deficiency in von Willebrand factor (VWF)-cleaving protease ADAMTS13. Current treatment is based on plasma infusion for congenital TTP, or plasma exchange, often in combination with immunosuppressive agents, for acquired TTP. These treatment methods are not always effective; therefore, new treatment methods are highly necessary. N-acetylcysteine (NAC), an FDA-approved anti-mucolytic agent, is a possible new treatment strategy for TTP, as it was demonstrated to reduce disulfide bonds in VWF, thereby decreasing VWF multimers size and hence their prothrombotic potential...
February 23, 2017: Blood
https://www.readbyqxmd.com/read/27991718/polycythemia-vera-and-essential-thrombocythemia-2017-update-on-diagnosis-risk-stratification-and-management
#18
Ayalew Tefferi, Tiziano Barbui
DISEASE OVERVIEW: Polycythemia Vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms respectively characterized by erythrocytosis and thrombocytosis; other disease features include leukocytosis, splenomegaly, thrombosis, bleeding, microcirculatory symptoms, pruritus, and risk of leukemic or fibrotic transformation. DIAGNOSIS: PV is defined by a JAK2 mutation, whose absence, combined with normal or increased serum erythropoietin level, makes the diagnosis unlikely...
January 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27922890/tolerability-and-biological-effects-of-long-acting-octreotide-in-patients-with-continuous-flow-left-ventricular-assist-devices
#19
Rajiv Malhotra, Keyur B Shah, Raveen Chawla, Sammy Pedram, Melissa C Smallfield, Anna G Priday, Christine T DeWilde, Donald F Brophy
Patients with implanted continuous, non-pulsatile, left ventricular assist devices (LVADs) have increased occurrence of gastrointestinal bleeding (GIB). While the pathophysiology is multifactorial, there are few treatments beyond supportive care. Octreotide acetate is a somatostatin analog that reduces GIB in various patient populations. However, there are sparse case-series that suggest octreotide acetate may reduce GIB in LVAD patients. This 10 patient, 28-week Phase I study evaluated the safety and tolerability of octreotide acetate long-acting release (LAR) 20 mg depot injection every four weeks until week 16 following LVAD placement...
December 2, 2016: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/27919526/factors-related-to-the-development-of-acquired-von-willebrand-syndrome-in-patients-with-essential-thrombocythemia-and-polycythemia-vera
#20
A Rottenstreich, G Kleinstern, S Krichevsky, D Varon, D Lavie, Y Kalish
OBJECTIVE: We characterized acquired von Willebrand syndrome (AVWS) among essential thrombocythemia (ET) and polycythemia vera (PV) patients. METHODS: A review of patients with ET or PV evaluated for AVWS. RESULTS: Of 116 patients with ET, 64 (55%) developed AVWS; of 57 with PV, 28 (49%) developed AVWS. Median platelet counts of ET and PV patients who developed AVWS were 920×10(9)/L and 679×10(9)/L, respectively (P=0.01). Of patients who developed AVWS, 69...
December 2, 2016: European Journal of Internal Medicine
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