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https://www.readbyqxmd.com/read/28634421/the-role-of-von-willebrand-factor-in-vascular-inflammation-from-pathogenesis-to-targeted-therapy
#1
REVIEW
Felice Gragnano, Simona Sperlongano, Enrica Golia, Francesco Natale, Renatomaria Bianchi, Mario Crisci, Fabio Fimiani, Ivana Pariggiano, Vincenzo Diana, Andreina Carbone, Arturo Cesaro, Claudia Concilio, Giuseppe Limongelli, Mariagiovanna Russo, Paolo Calabrò
Beyond its role in hemostasis, von Willebrand factor (VWF) is an emerging mediator of vascular inflammation. Recent studies highlight the involvement of VWF and its regulator, ADAMTS13, in mechanisms that underlie vascular inflammation and immunothrombosis, like leukocyte rolling, adhesion, and extravasation; vascular permeability; ischemia/reperfusion injury; complements activation; and NETosis. The VWF/ADAMTS13 axis is implicated in the pathogenesis of atherosclerosis, promoting plaque formation and inflammation through macrophage and neutrophil recruitment in inflamed lesions...
2017: Mediators of Inflammation
https://www.readbyqxmd.com/read/28628948/platelet-hyperaggregability-is-associated-with-decreased-adamts13-activity-and-enhanced-endotoxemia-in-patients-with-acute-cholangitis
#2
Hiroaki Takaya, Hideto Kawaratani, Takuya Kubo, Kenichiro Seki, Yasuhiko Sawada, Kosuke Kaji, Yasushi Okura, Kosuke Takeda, Mitsuteru Kitade, Kei Moriya, Tadashi Namisaki, Akira Mitoro, Masanori Matsumoto, Hiroshi Fukui, Hitoshi Yoshiji
AIM: Insufficient ADAMTS13 activity (ADAMTS13:AC) leads to increased levels of unusually large von Willebrand factor (VWF) multimers and causes microcirculatory disturbance and multiple organ failure (MOF). Endotoxin (Et) triggers the activation of coagulation and cytokine cascades, leading to MOF in severe inflammatory response syndrome (SIRS). Here, we investigated the potential role of endotoxemia-related ADAMTS13 in acute cholangitis. METHODS: Twenty-four patients with acute cholangitis, including 7 with severe acute cholangitis, were recruited in this study...
June 19, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28618874/von-willebrand-factor-multimeric-structure-and-functional-activity-in-patients-with-atrial-fibrillation-with-and-without-oral-anticoagulation
#3
Sandra Lopez-Castaneda, Ignacio Valencia-Hernández, Carlos Arean, Daniel Godínez-Hernández, Martha Eva Viveros-Sandoval
von Willebrand factor (vWF) is a multimeric glycoprotein present in blood plasma. It is synthesized in megakaryocytes and endothelial cells, secreted into circulation in the form of high-molecular-weight multimers (HMWMs), and cleaved into shorter, less active multimers by ADAMTS13. It is essential for platelet adhesion and aggregation. Previous studies have investigated the relationship between vWF levels and thromboembolic events with little regard to vWF multimeric structure. Patients with atrial fibrillation (AF) exhibit higher plasma vWF and lower ADAMTS13 levels...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28596255/thrombotic-thrombocytopenic-purpura-associated-with-pegylated-interferon-alfa-2a-use-in-a-patient-with-polycythemia-vera
#4
Radhika Gangaraju, Soo J Kim, Jing-Fei Dong, Sabina Swierczek, Josef T Prchal
Pegylated interferon alfa-2a (pegIFNa) is being increasingly used for treatment of myeloproliferative neoplasms; however, its side effects, including autoimmune complications, are not unusual. We report on a 47-year-old woman with polycythemia vera (PV) treated with pegIFNa and in complete hematologic remission who developed thrombotic thrombocytopenic purpura (TTP). To our knowledge, thrombotic microangiopathy has been reported as a side effect of interferon (IFN) use in patients with hepatitis and chronic myeloid leukemia, but not in those with PV...
June 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28596204/malignant-hypertension-as-a-rare-cause-of-thrombotic-microangiopathy
#5
Guramrinder Thind, Karthik Kailasam
Malignant hypertension can occasionally be associated with microangiopathic haemolytic anaemia. A 38-year-old male presented with nausea, vomiting, loss of appetite and oliguria for 2 weeks. He was diagnosed with hypertensive emergency with cardiac and renal dysfunction. Interestingly, further workup was diagnostic for the presence of thrombotic microangiopathy (TMA): haemoglobin =12.7 g/dL, indirect bilirubin =2.0 mg/dL, haptoglobin ≤6 mg/dL, platelet count =121 000/μL and schistocytes on peripheral smear...
June 8, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28591212/reduced-adamts13-levels-in-patients-with-acute-and-chronic-cerebrovascular-disease
#6
Frederik Denorme, Peter Kraft, Inge Pareyn, Christiane Drechsler, Hans Deckmyn, Karen Vanhoorelbeke, Christoph Kleinschnitz, Simon F De Meyer
Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is known about a correlation with ADAMTS13. Therefore, the aim of this study was to compare plasma levels of ADAMTS13 between 85 healthy volunteers (HV), 104 patients with acute ischemic stroke and 112 patients with a chronic cerebrovascular disease (CCD). In this case-control study, plasma ADAMTS13 antigen levels were measured by ELISA and plasma VWF levels, measured previously, were next used to calculate VWF:ADAMTS13 ratios...
2017: PloS One
https://www.readbyqxmd.com/read/28576877/presenting-adamts13-antibody-and-antigen-levels-predict-prognosis-in-immune-mediated-thrombotic-thrombocytopenic-purpura
#7
Ferras Alwan, Chiara Vendramin, Karen Vanhoorelbeke, Katy Langley, Vickie McDonald, Steve Austin, Amanda Clark, William Lester, Richard Gooding, Tina Biss, Tina Dutt, Nichola Cooper, Oliver Chapman, Tanya Cranfield, Kenny Douglas, H G Watson, J J van Veen, Keith Sibson, William Thomas, Lynn Manson, Quentin A Hill, Sylvia Benjamin, Debra Ellis, John-Paul Westwood, Mari Thomas, Marie Scully
Immune-mediated Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening disorder caused by antibodies against ADAMTS13. From the United Kingdom TTP registry we undertook a prospective study investigating the impact of the presenting anti-ADAMTS13 IgG antibody and ADAMTS13 antigen on mortality. 312 episodes involving 292 patients over 87 months were included. 68% were female, median age 46 (range 11-88 years) and median presenting ADAMTS13 of <5% (range <5% - 18%). The mortality rate was 10.3% (n=32)...
June 2, 2017: Blood
https://www.readbyqxmd.com/read/28550351/diagnostic-and-treatment-guidelines-for-thrombotic-thrombocytopenic-purpura-ttp-2017-in-japan
#8
Masanori Matsumoto, Yoshihiro Fujimura, Hideo Wada, Koichi Kokame, Yoshitaka Miyakawa, Yasunori Ueda, Satoshi Higasa, Takanori Moriki, Hideo Yagi, Toshiyuki Miyata, Mitsuru Murata
Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP...
July 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28547839/thrombotic-thrombocytopenic-purpura-related-to-adamts13-deficiency-and-successful-treatment-in-a-chimpanzee-pan%C3%A2-troglodytes-verus
#9
Hester van Bolhuis, Marno Wolters, Mark de Boer, Rob Fijnheer, Martine van Zijll Langhout, Henk Niphuis, Carel Eckmann
A 27-year-old male chimpanzee (Pan troglodytes verus) developed signs of thrombotic thrombocytopenic purpura (TTP). ADAMTS13 deficiency appeared to be the cause of disease. After treatment with high-dose prednisone, haematological values and clinical signs recovered. This is the first description of spontaneous TTP associated with ADAMTS13 deficiency in a non-human primate.
May 26, 2017: Journal of Medical Primatology
https://www.readbyqxmd.com/read/28523822/development-of-a-novel-flow-cytometric-immunobead-array-to-quantify-vwf-ag-and-vwf-gpibr-and-its-application-in-acute-myocardial-infarction
#10
Bin Yan, Mengqiao Xu, Yunxiao Zhao, Haijun Guo, Lijun Xia, Changgeng Ruan, Yiming Zhao
OBJECTIVES: Both von Willebrand disease (VWD) and acute myocardial infarction (AMI) involve quantitative and qualitative changes in von Willebrand factor (VWF). Our objective was to develop a rapid and precise flow cytometric immunobead array (FCIA) to quantify VWF antigen (VWF:Ag) and ristocetin-triggered platelet glycoprotein Ib binding (VWF:GPIbR) and apply it in a clinical setting. METHODS: Microbeads, coated with monoclonal antibodies for SZ29 or SZ151 IgG, were incubated with diluted plasma...
May 18, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28521259/von-willebrand-factor-and-its-cleaving-protease-adamts13-balance-in-coronary-artery-vessels-lessons-learned-from-thrombotic-thrombocytopenic-purpura-a-narrative-review
#11
REVIEW
Nuccia Morici, Silvia Cantoni, Francesco Panzeri, Alice Sacco, Chiara Rusconi, Miriam Stucchi, Fabrizio Oliva, Marco Cattaneo
BACKGROUND: Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a microangiopathic syndrome that presents as an acute medical emergency. In this review we will explore the evidence of a two-way relationship between TTP and ACS. Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS. METHODS AND RESULTS: Pubmed, MEDLINE and EMBASE, CINHAL, COCHRANE and Google Scholar databases were searched from inception to January 2017...
May 12, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28512098/a-rare-case-of-thrombotic-microangiopathy-triggered-by-acute-pancreatitis
#12
Kevin Singh, Ahmed Jamal Nadeem, Behzad Doratotaj
Thrombotic microangiopathy (TMA) occurring after acute pancreatitis is rarely described. Without prompt intervention, TMA can be, and often is, lethal, so prompt recognition is important. Here, we present a case of a 61-year-old woman with a history of alcohol misuse who presented with epigastric pain, nausea and vomiting after binge drinking. Elevated serum lipase and imaging were suggestive of acute-on-chronic pancreatitis. Although the patient's symptoms of acute pancreatitis subsided, her anaemia, thrombocytopenia and acute kidney injury worsened...
May 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#13
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28500622/implementation-of-a-rapid-assay-of-adamts13-activity-was-associated-with-improved-30-day-survival-rate-in-patients-with-acquired-primary-thrombotic-thrombocytopenic-purpura-who-received-platelet-transfusions
#14
Yumi Yoshii, Yoshihiro Fujimura, Charles L Bennett, Ayami Isonishi, Norio Kurumatani, Masanori Matsumoto
BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28499011/fatal-case-of-chikungunya-and-concomitant-thrombotic-thrombocytopenic-purpura-in-french-guiana-during-air-flight-medical-evacuation
#15
Loïc Epelboin, Bastien Bidaud, Emilie Mosnier, Paul Le Turnier, Guillaume Vesin, Gaëlle Walter, Philippe Abboud, Stéphanie Houcke, Maïa Forgues, Gérald Egmann, Alain Stepanian, Félix Djossou
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated to severe ADAMTS13 deficiency. It has been linked to various viral infections. Among arboviruses, only Crimean-Congo haemorrhagic fever and dengue fever have been linked to this severe disease. We report the first documented case of TTP concomitant to Chikungunya virus infection.
September 1, 2017: Journal of Travel Medicine
https://www.readbyqxmd.com/read/28495930/adamts13-retards-progression-of-diabetic-nephropathy-by-inhibiting-intrarenal-thrombosis-in-mice
#16
Nirav Dhanesha, Prakash Doddapattar, Mehul R Chorawala, Manasa K Nayak, Koichi Kokame, Janice M Staber, Steven R Lentz, Anil K Chauhan
OBJECTIVE: ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type I repeats-13) prevents microvascular thrombosis by cleaving prothrombogenic ultralarge von Willebrand factor (VWF) multimers. Clinical studies have found association between reduced ADAMTS13-specific activity, ultralarge VWF multimers, and thrombotic angiopathy in patients with diabetic nephropathy. It remains unknown, however, whether ADAMTS13 deficiency or ultralarge VWF multimers have a causative effect in diabetic nephropathy...
May 11, 2017: Arteriosclerosis, Thrombosis, and Vascular Biology
https://www.readbyqxmd.com/read/28480350/therapeutic-efficacy-of-the-platelet-glycoprotein-ib-antagonist-anfibatide-in-murine-models-of-thrombotic-thrombocytopenic-purpura
#17
Liang Zheng, Yingying Mao, Mohammad S Abdelgawwad, Nicole K Kocher, Mandy Li, Xiangrong Dai, Benjamin Li, X Long Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal blood clot disorder, is primarily caused by severe deficiency of plasma ADAMTS13 activity resulting from acquired autoantibodies. Plasma exchange is the only effective initial therapy. However, the high mortality rate and the complications associated with plasma exchange therapy remain a major concern. To address unmet clinical needs, therapeutic efficacies of anfibatide, a snake venom-derived platelet glycoprotein Ib antagonist, in murine models of spontaneous thrombocytopenia and shigatoxin-induced TTP were determined...
November 29, 2016: Blood Advances
https://www.readbyqxmd.com/read/28473932/acquired-thrombotic-thrombocytopenic-purpura-in-a-patient-with-pernicious-anemia
#18
Ramesh Kumar Pandey, Sumit Dahal, Kamal Fadlalla El Jack Fadlalla, Shambhu Bhagat, Bikash Bhattarai
Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28457019/dynamics-of-von-willebrand-factor-reactivity-in-sickle-cell-disease-during-vaso-occlusive-crisis-and-steady-state
#19
J W R Sins, M Schimmel, B M Luken, E Nur, S S Zeerleder, C F J van Tuijn, D P M Brandjes, W F Kopatz, R T Urbanus, J C M Meijers, B J Biemond, K Fijnvandraat
Essentials The role of von Willebrand Factor (VWF) in the pathophysiology of sickle cell disease is unclear. We assessed markers of VWF during admission for vaso-occlusive crisis (VOC) and steady state. VWF reactivity was higher during VOC and was associated with inflammation and neutrophil activation. Hyper-adhesive VWF may promote VOC in sickle cell disease. SUMMARY: Background Endothelial activation plays a central role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), facilitating adhesive interactions with circulating blood cells...
April 29, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28455885/an-extremely-rare-splice-site-mutation-in-the-gene-encoding-complement-factor-i-in-a-patient-with-atypical-hemolytic-uremic-syndrome
#20
Tina S Ipe, Jooeun Lim, Meredith Anne Reyes, Mike Ero, Christopher Leveque, Bradley Lewis, Jamey Kain
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure. The disease is difficult to diagnose due to its similarity with other hematologic disorders, such as thrombotic thrombocytopenic purpura (TTP). However, genetic mutations are found in 50-70% of patients with aHUS and can be useful in its diagnosis. STUDY DESIGN AND METHODS: A 40-year-old male presented to our hospital with acute kidney injury, evidenced by high creatinine levels (8...
April 28, 2017: Journal of Clinical Apheresis
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