Argininemia and vitamin K-dependent coagulation factors deficiency: A case report and a brief review of the literature
Arginase deficiency (AD) is caused by an autosomal recessive mutation in the gene coding for arginase 1 (ARG1). The estimated prevalence of AD is 1 per 950,000 births, which corresponds to around 3  % of all urea cycle disorders (UCDs) (1). After birth, UCDs result in the accumulation of ammonia; this causes neurologic impairments, liver disorders, gastrointestinal tract problems, and psychiatric disorders (1–3). Relative to other UCDs, the signs of AD are milder and start to appear later (1–3). Low blood levels of vitamin K-dependent coagulation factors (VKCFD) like FVII, FIX, FX and protein C have been reported, an...
Source: Thrombosis Research - June 29, 2023 Category: Hematology Authors: Tasnim Bel Hadj, Val érie Li Thiao Te, Maïlys Le Guyader, Annelise Voyer, Charlotte Durand-Maugard, Antoine Galmiche, Loïc Garçon, Julien Demagny Tags: Letter to the Editors-in-Chief Source Type: research

What do we know about platelets in myocardial ischemia-reperfusion injury and why is it important?
Myocardial ischemia-reperfusion injury (MIRI), the joint result of ischemic injury and reperfusion injury, is associated with poor outcomes in patients with acute myocardial infarction undergoing primary percutaneous coronary intervention. Accumulating evidence demonstrates that activated platelets directly contribute to the pathogenesis of MIRI through participating in the formation of microthrombi, interaction with leukocytes, secretion of active substances, constriction of microvasculature, and activation of spinal afferent nerves. (Source: Thrombosis Research)
Source: Thrombosis Research - June 29, 2023 Category: Hematology Authors: Lun Wang, Yifan Liu, Ran Tian, Wei Zuo, Hao Qian, Liang Wang, Xinglin Yang, Zhenyu Liu, Shuyang Zhang Tags: Review Article Source Type: research

Editorial Board
(Source: Thrombosis Research)
Source: Thrombosis Research - June 28, 2023 Category: Hematology Source Type: research

Plasma levels of coagulation factors VIII and IX and risk of venous thromboembolism: Systematic review and meta-analysis
The associations of plasma factor VIII (FVIII) and factor IX (FIX) levels with risk of venous thromboembolism (VTE) are not well defined. We performed a systematic review and meta-analysis of these associations. (Source: Thrombosis Research)
Source: Thrombosis Research - June 27, 2023 Category: Hematology Authors: Gordon Lowe, Olivia Wu, Astrid van Hylckama Vlieg, Aaron Folsom, Frits Rosendaal, Mark Woodward Tags: Full Length Article Source Type: research

Major cardiovascular events after COVID-19, event rates post-vaccination, antiviral or anti-inflammatory therapy, and temporal trends: Correspondence
We would like to share ideas on the publication “Bilate Major cardiovascular events after COVID-19, event rates post-vaccination, antiviral or anti-inflammatory therapy, and temporal trends: Rationale and methodology of the CORONA-VTE-Network study [1].” Kdeli et al. concluded that: The CORONA-VTE-Network study will share contemporary informa tion related to rates of cardiovascular and thrombotic events in patients with COVID-19 overall, as well as within key subgroups, including by time of inclusion, vaccination status, patients undergoing hemodialysis, the elderly, and sex-informed analyses such as comparison of wome...
Source: Thrombosis Research - June 26, 2023 Category: Hematology Authors: Amnuay Kleebayoon, Viroj Wiwanitkit Tags: Letter to the Editors-in-Chief Source Type: research

Cancer-associated thrombosis by cancer sites and inherited factors in a prospective population-based cohort
Cancer-associated thrombosis (CAT) is common and associated with mortality. We estimated CAT rate by cancer sites and inherited factors among cancer patients from the UK Biobank (N  = 70,406). The 12-month CAT rate after cancer diagnosis was 2.37 % overall but varied considerably among cancer sites. Among the 10 cancer sites classified as ‘high-risk’ of CAT by the National Comprehensive Cancer Network guidelines, 6 had CAT rate  5 %. (Source: Thrombosis Research)
Source: Thrombosis Research - June 26, 2023 Category: Hematology Authors: Zhuqing Shi, Jun Wei, Andrew S. Rifkin, Chi-Hsiung Wang, Liana K. Billings, Jonathan S.H. Woo, Mark S. Talamonti, Tilley Jenkins Vogel, Elena Moore, Bruce E. Brockstein, Janardan D. Khandekar, Henry M. Dunnenberger, Peter J. Hulick, David Duggan, S. Lilly Tags: Letter to the Editors-in-Chief Source Type: research

Long term follow-up of a multicentre cohort of COVID-19 patients with pulmonary embolism: Anticoagulation management and outcomes
Pulmonary embolism (PE) is a frequent complication in COVID19 hospitalized patients. Inflammatory storm and endothelial dysfunction due to the virus seem to be the two major risk factors for PE. Consequently, PE related to COVID19 could be consider as triggered by a transient inflammatory acute phase and treated for no longer than 3  months. However, few data are available on management of anticoagulation and risk of venous thromboembolic (VTE) recurrences in these patients and guidelines are still undefined. (Source: Thrombosis Research)
Source: Thrombosis Research - June 23, 2023 Category: Hematology Authors: Lucia Filippi, Gianni Turcato, Marta Milan, Sofia Barbar, Eliana Miozzo, Arian Zaboli, Diego Tonello, Daniela Milazzo, Massimo Marchetti, Stefano Cuppini, Paolo Prandoni Tags: Full Length Article Source Type: research

Hemostatic efficacy of four factor prothrombin complex concentrate in intracerebral hemorrhage patients receiving warfarin vs. factor Xa inhibitors
4-F PCC is administered for reversal of factor Xa inhibitor-associated coagulopathy despite a lack of quality evidence demonstrating hemostatic efficacy. The aim of this study was to evaluate the hemostatic efficacy of 4-F PCC in intracerebral hemorrhage patients who received factor Xa inhibitors versus warfarin. (Source: Thrombosis Research)
Source: Thrombosis Research - June 23, 2023 Category: Hematology Authors: Gregory Pon, Brittany Pelsue, Sujan T. Reddy, Kaushik Parsha, Xu Zhang, Brian Gulbis, Andrew Barreto, Sean I. Savitz, Miguel Escobar, Teresa A. Allison Tags: Full Length Article Source Type: research

Protein S deficiency caused by cryptic splicing due to the novel intron variant c.346+5G > C in PROS1
Protein S (PS) is a plasma single-chain glycoprotein encoded by the PS gene (PROS1). Although plasma PS is mainly synthesized by hepatocytes, it is also synthesized by megakaryocytes and vascular endothelial cells [1]. PS is a cofactor of activated protein C, and it exerts anticoagulant effects by promoting the inactivation of activated coagulation factor V and coagulation factor VIII [2]. Hereditary PS deficiency is a condition of thrombophilia caused by variants in the PROS1 gene, and it has an autosomal-dominant form of inheritance with incomplete penetrance [2,3]. (Source: Thrombosis Research)
Source: Thrombosis Research - June 23, 2023 Category: Hematology Authors: Satomi Nagaya, Tomoki Togashi, Masaharu Akiyama, Yuta Imai, Haruto Matsumoto, Haruka Moriya, Makiko Meguro-Horike, Ibuki Yasuda, Yuika Kikuchi, Yamato Kuwajima, Shin-ichi Horike, Atsushi Watanabe, Eriko Morishita Tags: Letter to the Editors-in-Chief Source Type: research

Direct oral anticoagulants for the treatment of splanchnic vein thrombosis – A systematic review and meta-analysis
Splanchnic vein thrombosis (SVT) is an uncommon manifestation of venous thromboembolism in the splanchnic venous system, with scarce evidence surrounding its management. We assessed the efficacy and safety of direct oral anticoagulant (DOAC) to low-molecular-weight heparins (LMWH), vitamin-k antagonists (VKAs), or no anticoagulation. (Source: Thrombosis Research)
Source: Thrombosis Research - June 20, 2023 Category: Hematology Authors: Allen Li, Ming Chan Zhang, Pei Li, Ali Eshaghpour, Katherine Li, Marc Carrier, Philip Wells, Mark Andrew Crowther Source Type: research

Demographic, clinical, and echocardiographic factors associated with residual perfusion defects beyond six months after pulmonary embolism
Residual perfusion defects (RPD) after pulmonary embolism (PE) are common. (Source: Thrombosis Research)
Source: Thrombosis Research - June 16, 2023 Category: Hematology Authors: Øyvind Jervan, Adam Dhayyat, Jostein Gleditsch, Stacey Haukeland-Parker, Mazdak Tavoly, Frederikus A. Klok, Diyar Rashid, Knut Stavem, Waleed Ghanima, Kjetil Steine Tags: Full Length Article Source Type: research

Sources of variability in the human platelet transcriptome
Platelets are anucleated cells produced by megakaryocytes, from which they inherit all the components necessary to carry their functions. They circulate in blood vessels where they play essential roles in coagulation, wound repair or inflammation, and have been implicated in various pathological conditions such as thrombosis, viral infection or cancer progression. The importance of these cells has been established over a century ago, and effective anti-platelet medications with different mechanisms of action have since been developed. (Source: Thrombosis Research)
Source: Thrombosis Research - June 16, 2023 Category: Hematology Authors: Florian Thibord, Andrew D. Johnson Source Type: research

Safety and efficacy of caplacizumab retreatment in a real-life monocentric cohort of patients with immune-mediated thrombotic thrombocytopenic purpura
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is an acute life-threatening disease characterized by the association of thrombocytopenia, microangiopathic hemolytic anemia, organ involvement and ADAMTS-13 protease deficiency [1]. The standard of care for iTTP has historically been based on therapeutic plasma exchange (TPE) and immune suppression. More recently, the treatment with caplacizumab has led to a significantly faster iTTP recovery. This is a nanobody that targets the interaction between platelet's glycoprotein 1b and the ultra-large A1 domain of von Willebrand factor (VWF). (Source: Thrombosis Research)
Source: Thrombosis Research - June 15, 2023 Category: Hematology Authors: G. Caocci, O. Mulas, D. Mantovani, N. Bandinu, G. La Nasa Tags: Letter to the Editors-in-Chief Source Type: research

Development of a predictive model of venous thromboembolism recurrence in anticoagulated cancer patients using machine learning
This study aimed to develop a predictive model to assess the risk of VTE recurrence within 6  months at the moment of primary VTE diagnosis in these patients. (Source: Thrombosis Research)
Source: Thrombosis Research - June 15, 2023 Category: Hematology Authors: Andres J. Mu ñoz, Juan Carlos Souto, Ramón Lecumberri, Berta Obispo, Antonio Sanchez, Jorge Aparicio, Cristina Aguayo, David Gutierrez, Andrés García Palomo, Victor Fanjul, Carlos del Rio-Bermudez, María Carmen Viñuela-Benéitez, Miguel Ángel Hern Tags: Full Length Article Source Type: research

Implementation of a clinical decision-making tool for perioperative management of vitamin K antagonists in patients with atrial fibrillation
Perioperative management of anticoagulant treatment, such as vitamin K antagonists (VKA) and direct oral anticoagulants (DOAC), is challenging. The risk of perioperative bleeding and thromboembolic complications is determined by both patient and procedure-related factors, thus the individual-based periprocedural management of oral anticoagulant treatment often requires a multidisciplinary approach. Previous guidelines recommended patients on VKA treatment at high risk of thromboembolic complications to receive low-molecular-weight heparin (LMWH) at therapeutic dose during the period of interruption of oral anticoagulant tr...
Source: Thrombosis Research - June 15, 2023 Category: Hematology Authors: Eva N. Hamuly ák, Dirk Westenberg, Carlinda Bresser, Simone Sissing, Marjan Ruiter, Luuk J.J. Scheres, Arian Wals, Laura M. Faber Tags: Letter to the Editors-in-Chief Source Type: research