Article
Hematology
Maria Teresa Pagliari, Ulrich Budde, Luciano Baronciani, Peyman Eshghi, Minoo Ahmadinejad, Zahra Badiee, Mohammad-Reza Baghaipour, Olga Benitez Hidalgo, Eugenia Biguzzi, Imre Bodo, Giancarlo Castaman, Jenny Goudemand, Mehran Karimi, Bijan Keikhaei, Riitta Lassila, Frank W. G. Leebeek, Maria Fernanda Lopez Fernandez, Renato Marino, Johannes Oldenburg, Ian Peake, Cristina Santoro, Reinhard Schneppenheim, Andreas Tiede, Gholamreza Toogeh, Alberto Tosetto, Marc Trossaert, Hamideh Yadegari, Eva M. K. Zetterberg, Pier Mannuccio Mannucci, Augusto B. Federici, Jeroen Eikenboom, Flora Peyvandi
Summary: The prevalence of anti-von Willebrand factor (VWF) antibodies was assessed in patients with type 3 von Willebrand disease (VWD). The study found that 8.4% of the subjects tested positive for anti-VWF antibodies, while 6% had neutralizing VWF inhibitors. These inhibitors were mainly found in patients homozygous for VWF null alleles.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Kayleigh M. Voos, Wenpeng Cao, Nicholas A. Arce, Emily R. Legan, Yingchun Wang, Asif Shajahan, Parastoo Azadi, Pete Lollar, Xiaohui Frank Zhang, Renhao Li
Summary: Desialylation of O-glycans around A1 increases its activity by destabilizing the AIM, leading to enhanced binding activity and platelet aggregation.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Soracha Ward, Jamie M. O'Sullivan, James S. O'Donnell
Summary: Glycosylation is a crucial posttranslational modification that plays a key role in regulating protein biology with variations in glycan structures implicated in human diseases. The heavily glycosylated von Willebrand factor (VWF) with N- and O-linked glycans affecting its structure, function, and half-life, particularly highlighting the significant role of O-linked glycans in modulating VWF biology.
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2021)
Article
Hematology
Cornelia Englisch, Florian Moik, Stephan Nopp, Markus Raderer, Ingrid Pabinger, Cihan Ay
Summary: Non-O blood type is associated with increased VTE risk in cancer patients, particularly after 3 months and in patients with intermediate and low-risk tumor types.
Review
Hematology
Salah Elsherif, Ali Zidan, Olivia Saville, Maha Othman
Summary: Cancer-associated thrombosis, especially venous thromboembolism, is associated with ABO blood groups, with non-O blood type and increased VWF levels showing a strong correlation with increased VTE risk. However, there are varying results in different studies and more research is needed to understand the role of ABO blood groups and other risk factors in different cancer types.
SEMINARS IN THROMBOSIS AND HEMOSTASIS
(2023)
Article
Hematology
Justine H. Ryu, Kenneth A. Bauer, Sol Schulman
Summary: This report describes the successful perioperative management of a patient with type 2N VWD using the recombinant FVIII product efanesoctocog alfa. By decoupling the FVIII-VWF interaction, efanesoctocog alfa achieves prolonged FVIII circulation and mitigates potential cardiovascular risks.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Biochemistry & Molecular Biology
Marcela Montilla, Isabel Atienza-Navarro, Francisco Jose Garcia-Cozar, Carmen Castro, Francisco Javier Rodriguez-Martorell, Felix A. Ruiz
Summary: Polyphosphate (polyP) plays an important modulatory role in the binding of von Willebrand factor (VWF) and Factor VIII (FVIII) in blood coagulation, and may be used in developing new therapies to activate VWF.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Hematology
Maria Teresa Pagliari, Frits R. Rosendaal, Minoo Ahmadinejad, Zahra Badiee, Mohammad-Reza Baghaipour, Luciano Baronciani, Olga Benitez Hidalgo, Imre Bodo, Ulrich Budde, Giancarlo Castaman, Peyman Eshghi, Jenny Goudemand, Mehran Karimi, Bijan Keikhaei, Riitta Lassila, Frank W. G. Leebeek, Maria Fernanda Lopez Fernandez, Pier Mannuccio Mannucci, Renato Marino, Johannes Oldenburg, Ian Peake, Cristina Santoro, Reinhard Schneppenheim, Andreas Tiede, Gholamreza Toogeh, Alberto Tosetto, Marc Trossaert, Hamideh Yadegari, Eva M. K. Zetterberg, Flora Peyvandi, Augusto B. Federici, Jeroen Eikenboom
Summary: The VWFpp/VWF:Ag ratio indicates missense variants, while the FVIII:C/VWF:Ag ratio does not discriminate between missense and null alleles. The level of VWFpp is not associated with the severity of bleeding symptoms.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Medicine, General & Internal
Aurore Collet, Christophe Zawadzki, Emmanuelle Jeanpierre, Caroline Kitel, Sylvain Dubucquoi, Eric Hachulla, Sophie Susen, David Launay
Summary: Systemic sclerosis (SSc) is associated with endothelial activation and fibrosis. Non-O blood group is linked to higher risk of thrombosis, fibrosis and autoimmune diseases. The distribution of ABO groups in SSc patients is similar to the general population, but non-O groups are associated with inflammation and endothelial activation, and a slightly higher frequency of pulmonary and vascular complications in SSc.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Medicine, Research & Experimental
John P. Reilly, Nuala J. Meyer, Michael G. S. Shashaty, Brian J. Anderson, Caroline Ittner, Thomas G. Dunn, Brian Lim, Caitlin Forker, Michael P. Bonk, Ethan Kotloff, Rui Feng, Edward Cantu, Nilam S. Mangalmurti, Carolyn S. Calfee, Michael A. Matthay, Carmen Mikacenic, Keith R. Walley, James Russell, David C. Christiani, Mark M. Wurfel, Paul N. Lanken, Muredach P. Reilly, Jason D. Christie
Summary: The study identified a replicable association between ABO blood type A1 and risk of ARDS among critically ill patients, possibly mediated through microvascular dysfunction and coagulation.
JOURNAL OF CLINICAL INVESTIGATION
(2021)
Review
Hematology
Omid Seidizadeh, Flora Peyvandi, Pier Mannuccio Mannucci
Summary: Von Willebrand disease is caused by quantitative or qualitative defects of von Willebrand factor (VWF). Type 2N VWD is a rare recessive disorder that requires accurate diagnosis and differentiation from other bleeding disorders such as mild/moderate hemophilia A.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
Stephen J. X. Murphy, Soon Tjin Lim, Fionnuala Hickey, Justin A. Kinsella, Deirdre R. Smith, Sean Tierney, Bridget Egan, T. Martin Feeley, Sinead M. Murphy, D. Ronan Collins, Tara Coughlan, Desmond O'Neill, Joseph A. Harbison, Prakash Madhavan, Sean M. O'Neill, Mary-Paula Colgan, James S. O'Donnell, Jamie M. O'Sullivan, George Hamilton, Dominick J. H. McCabe
Summary: The study found that VWF:Ag levels were higher and VWFpp/VWF:Ag ratios were lower in early symptomatic patients with carotid stenosis compared to asymptomatic patients, and these levels decreased over time. Additionally, there was a difference in VWF:Ag levels between patients with blood group O and those without blood group O.
THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
Maximilian Tscharre, Franziska Wittmann, Daniela Kitzmantl, Thomas Schloglhofer, Philip Cichra, Silvia Lee, Beate Eichelberger, Patricia P. Wadowski, Guenther Laufer, Dominik Wiedemann, Simon Panzer, Daniel Zimpfer, Thomas Gremmel
Summary: In this study, it was found that patients with blood group O who underwent LVAD implantation had a higher risk of bleeding, potentially due to lower levels of VWF and FVIII.
THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Biology
Klaus Bonazza, Roxana E. Iacob, Nathan E. Hudson, Jing Li, Chafen Lu, John R. Engen, Timothy A. Springer
Summary: This study investigates the regulatory role of N-linker and C-linker in the binding of A1 to GPIb alpha. The results show that the N-linker decreases the affinity of A1 for GPIb alpha, with a stronger contribution from its O-glycan moiety. Additionally, the N-linker regulates the hydrogen deuterium exchange, thermal stability, and urea-induced unfolding of A1. In contrast, the C-linker has no significant effect on the affinity of A1 for GPIb alpha.
Article
Hematology
Masayuki Kubo, Kazuya Sakai, Masaki Hayakawa, Hirokazu Kashiwagi, Hideo Yagi, Yoshinobu Seki, Atsushi Hasegawa, Haruyuki Tanaka, Itsuto Amano, Yoshiaki Tomiyama, Masanori Matsumoto
Summary: This study investigated whether VWF degradation is enhanced in patients with ET. The results showed that patients with higher platelet counts had lower HMW-VWFM index and higher VWF-DP:VWF Ag ratio. Cytoreductive therapy increased the HMW-VWFM index and decreased the VWF-DP:VWF Ag ratio. In conclusion, an increased platelet count in ET patients is associated with enhanced cleavage of VWF, and cytoreductive therapy can reduce this cleavage and improve VWFM distributions.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Alessandra Casonato, Elena Pontara, Margherita Morpurgo, Francesca Sartorello, Philip G. De Groot, Maria G. Cattini, Viviana Daidone, Luigi De Marco
BRITISH JOURNAL OF HAEMATOLOGY
(2015)
Article
Hematology
Viviana Daidone, Giorgia Saga, Giovanni Barbon, Elena Pontara, Maria G. Cattini, Margherita Morpurgo, Giuseppe Zanotti, Alessandra Casonato
BRITISH JOURNAL OF HAEMATOLOGY
(2015)
Article
Hematology
A. Casonato, M. G. Cattini, G. Barbon, V. Daidone, E. Pontara
THROMBOSIS RESEARCH
(2015)
Article
Hematology
V. Daidone, G. Barbon, M. G. Cattini, E. Pontara, C. Romualdi, I. Di Pasquale, K. Hosokawa, A. Casonato
Article
Multidisciplinary Sciences
Alessandra Casonato, Maria Grazia Cattini, Viviana Daidone, Elena Pontara, Antonella Bertomoro, Paolo Prandoni
Article
Hematology
Elena Pontara, Paolo Gresele, Maria Grazia Cattini, Viviana Daidone, Giovanni Barbon, Antonio Girolami, Ezio Zanon, Alessandra Casonato
BLOOD COAGULATION & FIBRINOLYSIS
(2014)
Article
Multidisciplinary Sciences
Raffaella Colombatti, Emiliano De Bon, Antonella Bertomoro, Alessandra Casonato, Elena Pontara, Elisabetta Omenetto, Graziella Saggiorato, Agostino Steffan, Tamara Damian, Giuseppe Cella, Simone Teso, Renzo Manara, Patrizia Rampazzo, Giorgio Meneghetti, Giuseppe Basso, Maria Teresa Sartori, Laura Sainati
Article
Hematology
Alessandra Casonato, Elena Pontara, Monica Battiston, Margherita Morpurgo, Maria Grazia Cattini, Elisabetta Casarin, Giorgia Saga, Viviana Daidone, Luigi De Marco
THROMBOSIS AND HAEMOSTASIS
(2013)
Article
Hematology
Viviana Daidone, Giovanni Barbon, Elena Pontara, Grazia M. Cattini, Lisa Gallinaro, Enrico Zampese, Paola Pizzo, Alessandra Casonato
THROMBOSIS AND HAEMOSTASIS
(2014)
Article
Hematology
Elena Pontara, Maria Grazia Cattini, Chunyan Cheng, Elisa Bison, Gentian Denas, Vittorio Pengo
Summary: This study indicates that aPS/PT antibodies have a strong anticoagulant effect, while a β2GPI antibodies exhibit mild anticoagulant and moderate procoagulant effects in thrombin generation and moderate aPC resistance.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Medicine, General & Internal
Alida Linda Patrizia Caforio, Giacomo De Luca, Anna Baritussio, Mara Seguso, Nicoletta Gallo, Elisa Bison, Maria Grazia Cattini, Elena Pontara, Luna Gargani, Alessia Pepe, Corrado Campochiaro, Mario Plebani, Sabino Iliceto, Giovanni Peretto, Antonio Esposito, Lorenzo Tofani, Alberto Moggi-Pignone, Lorenzo Dagna, Renzo Marcolongo, Marco Matucci-Cerinic, Cosimo Bruni
Summary: The study found that the frequencies of AHA and AIDA in SSc were significantly higher compared to other cardiac disease groups and normal blood donors, suggesting a high burden of underdiagnosed autoimmune heart involvement in SSc. AHA was associated with interstitial lung disease, chest pain history, abnormal troponin, AIDA, and current immunosuppression, as well as death and overall cardiac events during follow-up.
Article
Rheumatology
Chunyan Cheng, Elisa Bison, Elena Pontara, Maria Grazia Cattini, Marta Tonello, Gentian Denas, Vittorio Pengo
Summary: This study investigated microparticles (MPs) in patients with antiphospholipid antibodies (aPL) and found that the levels of total MPs and endothelial microparticles (EMPs) were significantly higher in triple positive patients compared to single positive patients, and were correlated with IgG antibody titers. Additionally, platelet microparticles (PMPs) were found to be the lowest in triple positive patients and inversely correlated with IgG antibody titers.
Article
Medicine, General & Internal
Anna Baritussio, Chun-yan Cheng, Giulia Lorenzoni, Cristina Basso, Stefania Rizzo, Monica De Gaspari, Francesco Fachin, Andrea Silvio Giordani, Honoria Ocagli, Elena Pontara, Maria Grazia Peloso Cattini, Elisa Bison, Nicoletta Gallo, Mario Plebani, Giuseppe Tarantini, Sabino Iliceto, Dario Gregori, Renzo Marcolongo, Alida Linda Patrizia Caforio
Summary: The role of inflammation markers in myocarditis is unclear. This study found that elevated CRP at diagnosis can identify patients with clinically suspected myocarditis and less severe clinical features, but does not contribute to predicting survival. The strongest predictors of death/heart transplant are reduced LVEF, biopsy-proven diagnosis, and positive ANA.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Hematology
Elena Pontara, Elisa Bison, Maria Grazia Cattini, Marta Tonello, Gentian Denas, Vittorio Pengo
Summary: In individuals who are tetra-positive for antiphospholipid antibodies, there is a significant correlation between total aPS/PT titers, LAC potency, and aPC-R. This suggests an interdependence between these parameters in tetra-positive subjects.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Viviana Daidone, Elena Pontara, Francesca Boscaro, Maria G. Cattini, Marta Milan, Alessandra Casonato
BLOOD COAGULATION & FIBRINOLYSIS
(2017)