Article
Obstetrics & Gynecology
H. M. G. Wiegers, E. N. Hamulyak, S. E. Damhuis, J. R. van Duuren, S. Darwish Murad, L. J. J. Scheres, S. J. Gordijn, J. Leentjens, J. J. Duvekot, M. N. Lauw, B. A. Hutten, S. Middeldorp, W. Ganzevoort
Summary: Women previously diagnosed with Budd-Chiari syndrome and/or portal vein thrombosis, with or without concomitant portal hypertension, can achieve relatively good pregnancy outcomes, with a high rate of term live births and lower risk of maternal and neonatal complications. Therefore, these conditions should not be considered as absolute contraindications for pregnancy, and individualized counseling and multidisciplinary pregnancy surveillance are essential.
BJOG-AN INTERNATIONAL JOURNAL OF OBSTETRICS AND GYNAECOLOGY
(2022)
Review
Medicine, General & Internal
Juan Carlos Garcia-Pagan, Dominique-Charles Valla
Summary: Primary Budd-Chiari Syndrome (BCS) is a life-threatening disease caused by obstruction of the hepatic venous outflow tract due to thrombosis or a primary venous wall disease. Recent advancements have enhanced our knowledge of BCS and its outcomes.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Review
Biochemistry & Molecular Biology
Vassilios P. Memtsas, Deepa R. J. Arachchillage, Diana A. Gorog
Summary: This review article discusses the pathophysiological role and laboratory assessment of fibrin, factor XIII, and endogenous fibrinolysis in the final phase of coagulation cascade and fibrinolysis, as well as their involvement in various disease states and assessment of cardiovascular risk with the most up-to-date evidence.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cardiac & Cardiovascular Systems
Wenzhu Li, Chase W. Kessinger, Makoto Orii, Hang Lee, Lang Wang, Ido Weinberg, Michael R. Jaff, Guy L. Reed, Peter Libby, Ahmed Tawakol, Peter K. Henke, Farouc A. Jaffer
Summary: The study found that RBF can reduce thrombus burden parameters and vein wall fibrosis and inflammation in mice on day 4 but not later. In the clinical study, PCDT demonstrated maximal benefits in improving quality of life and symptom scores for patients with symptom-onset-to-randomization timeframes of 4 to 8 days.
Review
Gastroenterology & Hepatology
Alberto Zanetto, Patrick Northup, Lara Roberts, Marco Senzolo
Summary: Hospitalised patients with decompensated cirrhosis experience a rebalanced haemostasis, but this balance can easily be disrupted, leading to bleeding. Acute kidney injury, bacterial infections/sepsis, and progression to acute-on-chronic liver failure further increase the risk of bleeding. Unfortunately, there is no single laboratory method to accurately assess bleeding risk and guide preventive measures. Understanding the haemostatic alterations during acute illness could improve the management of these patients.
JOURNAL OF HEPATOLOGY
(2023)
Article
Multidisciplinary Sciences
Yonghua Bi, Zhengyang Wu, Mengfei Yi, Xinwei Han, Jianzhuang Ren
Summary: In this study, recanalization of complicated inferior vena cava (IVC) occlusion in Budd-Chiari syndrome (BCS) patients was successfully achieved via the jugular-femoral venous (JFV) route. The technical success rate was high and the procedure was safe and effective, leading to clinical cure and improvement in a majority of patients.
SCIENTIFIC REPORTS
(2022)
Article
Biochemistry & Molecular Biology
Robin Forgacs, Gergely Peter Bokretas, Zoltan Monori, Zsolt Molnar, Zoltan Ruszkai
Summary: COVID-19-associated coagulopathy is a life-threatening complication, and optimal criteria for assessing the highest-risk patients are still unclear. Bedside point-of-care viscoelastic testing is a promising tool for recognizing coagulopathy, supporting treatment decisions, and monitoring efficacy.
Article
Medicine, General & Internal
Yuji Suzuki, Akiko Suzuki, Keisuke Kakisaka, Yasuhiro Takikawa
Summary: Acute Budd-Chiari syndrome and complete portal vein thrombosis, common features of myeloproliferative neoplasm, can lead to acute liver failure simultaneously. Liver transplantation can be an effective treatment option in this catastrophic condition, with low post-transplant complications.
CLINICAL CASE REPORTS
(2021)
Article
Hematology
Wiktoria Kuczmik, Waldemar E. Wysokinski, Gina K. Hesley, Danielle T. Vlazny, Damon E. Houghton, Keith E. Swanson, Ana Casanegra, David Hodge, Launia White, Robert D. McBane
Summary: This study aimed to compare clinical features and outcomes among patients with calf deep vein thrombosis (DVT) limited to the muscular veins and axial veins. The results showed that axial DVT patients are more likely to have a pulmonary embolism (PE) and experience recurrent venous thromboembolism (VTE), especially if anticoagulation is withheld.
THROMBOSIS AND HAEMOSTASIS
(2021)
Article
Hematology
Ellen G. Driever, Marta Magaz, Jelle Adelmeijer, Fanny Turon, Anna Baiges, Pol Olivas, Valeria Perez-Campuzano, Virginia Hernandez-Gea, Annabel Blasi, Juan-Carlos Garcia-Pagan, Ton Lisman
Summary: This study aimed to determine whether the portal vein (PV) has specific proinflammatory/hypercoagulable characteristics by comparing plasma sampled in the PV, hepatic vein (HV), and the systemic circulation. The results showed that although there were slightly higher levels of inflammatory markers in the PV, there was no clear proinflammatory or prothrombotic environment in the PV of cirrhotic patients. Multiple markers were found to be lower in the HV compared to the PV and systemic circulation, indicating that the liver plays a role in the clearance of these markers.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2022)
Article
Hematology
Siyu Jiang, Yingjie Ai, Xiaowen Fan, Xiaoquan Huang, Ling Wu, Liyuan Ni, Feng Li, Shiyao Chen
Summary: The aim of this study was to investigate the role of factor VIII (FVIII) in the occurrence of portal vein thrombosis (PVT) in cirrhotic patients with gastroesophageal variceal bleeding. The study found that elevated FVIII activity was associated with the occurrence and severity of PVT in these patients, suggesting that it may be a useful marker for identifying those at risk of PVT.
THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Microbiology
Xin-yu Huang, Ying-hui Zhang, Shi-yu Yi, Lei Lei, Tao Ma, Rui Huang, Lan Yang, Zhen-mao Li, Di Zhang
Summary: This study investigated the composition of gut microbiota and its role in the development of portal vein thrombosis (PVT) in liver cirrhosis (LC) patients. The results showed that LC patients with PVT had higher diversity and different structure of gut microbiota compared to those without PVT. The altered microbiota structure also led to differences in gut microbiota metabolism and was correlated with coagulation factor parameters. Animal experiments suggested that modulation of the gut microbiota, especially Bacteroides, could potentially reduce the progression of PVT in LC patients.
FRONTIERS IN MICROBIOLOGY
(2023)
Article
Gastroenterology & Hepatology
Anna Baiges, Bogdan Procopet, Gilberto Silva-Junior, Elba Llop, Luis Tellez, Anna Darnell, Angeles Garcia-Criado, Fanny Turon, Oana Nicoara-Farcau, Carlos Gonzalez-Alayon, Helene Larrue, Marta Magaz, Pol Olivas, Valeria Perez-Campuzano, Jose Luis Calleja, Agustin Albillos, Juan Carlos Reverter, Christophe Bureau, Jaime Bosch, Virginia Hernandez-Gea, Juan Carlos Garcia-Pagan
Summary: The aim of this study was to describe the incidence of recurrent thrombosis (RT) in patients with non-cirrhotic splanchnic vein thrombosis (NC-SVT) and identify its risk factors. The study found that the risk of RT in NC-SVT patients without an indication for long-term anticoagulation is not low. Furthermore, factor VIII level may be a key indicator for predicting RT.
JOURNAL OF HEPATOLOGY
(2023)
Article
Multidisciplinary Sciences
Xiaoju Hu, Xiaosen Jiang, Jia Li, Ni Zhao, Hairun Gan, Xinyan Hu, Luting Li, Xingtao Liu, Hong Shan, Yong Bai, Pengfei Pang
Summary: This study used whole-exome sequencing to identify potential risk mutations for Budd-Chiari syndrome (BCS) and developed a polygenic risk scoring model for BCS prediction. The study also revealed genetic risk differences between China and the West and identified shared genetic risks among BCS, venous thromboembolism, and vascular malformations.
Article
Cardiac & Cardiovascular Systems
Claire S. Whyte, Gael B. Morrow, Julia S. Gauer, Samantha J. Montague, Philip L. R. Nicolson
Summary: The 2023 annual meeting of the British Society for Haemostasis and Thrombosis (BSHT) was held in Birmingham, United Kingdom, focusing on novel therapeutics and emerging technology. This article discusses the exciting research presented at the meeting.
FRONTIERS IN CARDIOVASCULAR MEDICINE
(2023)
Article
Hematology
Greta Mulders, Hanneke van Verseveld, Joyce van der Geer, Eppo Wolvius, Frank Leebeek
Summary: This study investigates the oral health status of adult patients with haemophilia and finds that most patients have good oral health, but a few may experience bleeding problems during dental care and treatment.
Article
Hematology
Annette von Drygalski, Esteban Gomez, Adam Giermasz, Giancarlo Castaman, Nigel S. Key, Susan U. Lattimore, Frank W. G. Leebeek, Wolfgang A. Miesbach, Michael Recht, Robert Gut, Ricardo Dolmetsch, Paul E. Monahan, Sandra Le Quellec, Steven W. Pipe
Summary: This study reports the 3-year outcomes of using Etranacogene dezaparvovec in the treatment of adults with hemophilia B. The results show that Etranacogene dezaparvovec significantly increases FIX activity, reduces bleeding frequency, improves joint health, and has no major adverse events over the 3-year period.
Article
Hematology
Marieke J. A. Verhagen, Waander L. van Heerde, Johanna G. van der Bom, Erik A. M. Beckers, Nicole M. A. Blijlevens, Michiel Coppens, Samantha C. Gouw, Joop H. Jansen, Frank W. G. Leebeek, Lize F. D. van Vulpen, Danielle Meijer, Saskia E. M. Schols
Summary: The association between clinical bleeding phenotype and thrombin generation and plasmin generation profiles in patients with hemophilia was investigated. The study found that thrombin generation and plasmin generation parameters differed between patients with hemophilia and healthy individuals, and a decreased thrombin generation profile was associated with a severe clinical bleeding phenotype.
RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Eleonora Camilleri, Nienke van Rein, Bart J. M. van Vlijmen, Joseph S. Biedermann, Marieke J. H. A. Kruip, Frank W. Leebeek, Felix J. van der Meer, Christa M. Cobbaert, Suzanne C. Cannegieter, Willem M. Lijfering
Summary: The study found that rosuvastatin reduces coagulation factor levels through mechanisms related to the synthesis or regulation pathways of apolipoproteins.
RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Frank W. G. Leebeek, Flora Peyvandi, Andreas Tiede, Giancarlo Castaman, Miguel Escobar, Michael Wang, Bulent Zulfikar, Sophie Susen, Wolfgang Miesbach, Scarlett Wang, Yi Wang, Jingmei Zhang, Gulden Ozen
Summary: This study demonstrates the efficacy and safety of recombinant von Willebrand factor (rVWF) prophylaxis in patients with type 3 von Willebrand disease (VWD). The results show that rVWF prophylaxis significantly reduces bleeding rates in patients previously treated with on-demand VWF therapy or plasma-derived VWF prophylaxis. These findings highlight the effectiveness and safety of rVWF prophylaxis in type 3 VWD patients.
EUROPEAN JOURNAL OF HAEMATOLOGY
(2023)
Article
Medicine, General & Internal
S. W. Pipe, F. W. G. Leebeek, M. Recht, N. S. Key, G. Castaman, W. Miesbach, S. Lattimore, K. Peerlinck, P. Van der Valk, M. Coppens, P. Kampmann, K. Meijer, N. O'Connell, K. J. Pasi, D. P. Hart, R. Kazmi, J. Astermark, C. R. J. R. Hermans, R. Klamroth, R. Lemons, N. Visweshwar, A. von Drygalski, G. Young, S. E. Crary, M. Escobar, E. Gomez, R. Kruse-Jarres, D. V. Quon, E. Symington, M. Wang, A. P. Wheeler, R. Gut, Y. P. Liu, R. E. Dolmetsch, D. L. Cooper, Y. Li, B. Goldstein, P. E. Monahan
Summary: Etranacogene dezaparvovec gene therapy was found to be superior to prophylactic factor IX treatment in patients with hemophilia B with regards to the annualized bleeding rate, and it demonstrated a favorable safety profile.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Hematology
Roger E. G. Schutgens, Victor Jimenez-Yuste, Miguel Escobar, Anna Falanga, Bruna Gigante, Robert Klamroth, Riitta Lassila, Frank W. G. Leebeek, Michael Makris, Tarek Owaidah, Michelle Sholzberg, Andreas Tiede, David J. Werring, H. Bart van der Worp, Jerzy Windyga, Giancarlo Castaman
Summary: Cardiovascular disease is becoming more common in patients with hemophilia, and managing the delicate balance between thrombosis and hemostasis in these patients requires careful consideration. Specific recommendations are lacking for patients with very low clotting factor levels, and treatment strategies are mainly based on anecdotal evidence. For patients with higher clotting factor levels, antithrombotic therapy can be used with careful monitoring.
Article
Hematology
Martijn R. Brands, Lotte Haverman, Jelmer J. Muis, Mariette H. E. Driessens, Felix J. M. van der Meer, Geertje Goedhart, Stephan Meijer, Marianne de Jong, Johanna G. van der Bom, Marjon H. Cnossen, Karin Fijnvandraat, Samantha C. Gouw
Summary: This study examined the satisfaction of hemophilia patients in the Netherlands with their care and found high levels of satisfaction, but also identified areas for improvement such as better information provision and care coordination, with the potential use of digital health tools. These findings are important for further enhancing the care of hemophilia patients.
RESEARCH AND PRACTICE IN THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Pharmacology & Pharmacy
Sjoerd F. Koopman, Tine M. H. J. Goedhart, Laura H. Bukkems, Trevor M. Mulders, Frank W. G. Leebeek, Karin Fijnvandraat, Michiel Coppens, Mary Mathias, Peter W. Collins, R. Campbell Tait, Catherine N. Bagot, Nicola Curry, Jeanette Payne, Pratima Chowdary, Marjon H. Cnossen, Ron A. A. Mathot
Summary: This study evaluated the predictive performance of a population pharmacokinetic (PK) model for a recombinant factor IX Fc fusion protein (rFIX-Fc) and developed a new model using real-world data. The published model significantly underpredicted FIX activity levels, while the new model better described rFIX-Fc PK, especially for children aged <12 years.
BRITISH JOURNAL OF CLINICAL PHARMACOLOGY
(2023)
Article
Hematology
Martijn R. Brands, Ebony A. M. Janssen, Marjon H. Cnossen, Cees Smit, Lize F. D. Van Vulpen, Paul R. Van der Valk, Jeroen Eikenboom, Floor C. J. I. Heubel-Moenen, Louise Hooimeijer, Paula Ypma, Laurens Nieuwenhuizen, Michiel Coppens, Saskia E. M. Schols, Britta A. P. Laros-Van Gorkom, Frank W. G. Leebeek, Mariette H. E. Driessens, Frits R. Rosendaal, Johanna G. Van der Bom, Karin Fijnvandraat, Samantha C. Gouw
Summary: Transition readiness among Dutch adolescents and young adults with haemophilia was assessed, and it was found that poor treatment adherence and lack of preparation for healthcare costs can affect transition readiness. Improvements in team acquaintance and healthcare cost management can enhance transition readiness.
Editorial Material
Hematology
Frank Leebeek
LANCET HAEMATOLOGY
(2023)
Article
Hematology
Maurice Swinkels, Sophie Hordijk, Petra E. Buergisser, Johan A. Slotman, Tom Carter, Frank W. G. Leebeek, A. J. Gerard Jansen, Jan Voorberg, Ruben Bierings
Summary: This study investigated the release of VWF and VWFpp from individual alpha-granules in response to different physiological stimuli. The results showed that the secretion of alpha-granule cargo increased in a dose-dependent manner upon activation of PAR-1 and GPVI signaling pathways. However, there were differential release patterns for VWF and VWFpp.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
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
Eva K. Kempers, Calvin B. van Kwawegen, Joke de Meris, Manon C. W. Spaander, Saskia E. M. Schols, Paula F. Ypma, Floor C. J. I. Heubel-Moenen, Lize F. D. van Vulpen, Michiel Coppens, Johanna G. van der Bom, Karin Fijnvandraat, Karina Meijer, Jeroen Eikenboom, Samantha C. Gouw, Frank W. G. Leebeek, Marieke J. H. A. Kruip
Summary: The study evaluates the performance of FIT in patients with Von Willebrand disease (VWD) or hemophilia. The false-positive rate of FIT is higher in these patients compared to the general population. However, the detection rate of colorectal cancer (CRC) and/or advanced adenoma (AA) is high in hemophilia patients.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
Article
Hematology
Viola J. F. Strijbis, Lorenzo G. R. Romano, Ka Lei Cheung, Jeroen Eikenboom, Ying Poi Liu, Andrew C. McCreary, Frank W. G. Leebeek, Mettine H. A. Bos
Summary: This study aimed to evaluate the efficacy of FIX-FIAV in plasma from HA patients. The results showed that FIX-FIAV could increase the FVIII-equivalent activity and coagulation activity in HA patient plasma, mitigating the HA phenotype. Therefore, FIX-FIAV could serve as a potential treatment for HA patients.
JOURNAL OF THROMBOSIS AND HAEMOSTASIS
(2023)
