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External Quality Assessment Data for Investigation of von Willebrand Disease: Focus on Relative Utility of Contemporary Functional von Willebrand Factor Assays. The United Kingdom National External Quality Assessment Scheme (UK NEQAS) Experience
We report here findings from recent external quality assessment (EQA) exercises, as well as from a questionnaire about diagnostic practices employed by centers in the United Kingdom National Quality Assessment Scheme (UK NEQAS) performing von Willebrand factor (VWF) assays. Plasma samples from donors with VWD, “normal” donors, the International Society for Thrombosis and Haemostasis Scientific Subcommittee (ISTH SSC) plasma standard, and whole blood samples were sent to participants in the UK NEQAS BC program for VWF investigation. Calibration of lot#5 of the ISTH SSC plasma standard was shown to give improved comparab...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Jennings, Ian Reilly-Stitt, Chris Lowe, Anna Kitchen, Steve Walker, Isobel Tags: Review Article Source Type: research

How Do Laboratories Perform von Willebrand Disease Diagnostics and Classification of von Willebrand Disease Patients? Results from External Quality Data and an International Survey
Conclusions Diagnosis of VWD is heterogeneous in diagnostic approach, guidelines, and cutoff values within large ranges of VWF results between laboratories. Harmonization of approaches and increased accuracy of VWF measurements may help to establish a correct diagnosis. [...] Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Abstract  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Hollestelle, Martine J. Meijers, Joost C. M. Meijer, Piet Tags: Review Article Source Type: research

Thromboelastography-Guided Therapy Enhances Patient Blood Management in Cirrhotic Patients: A Meta-analysis Based on Randomized Controlled Trials
This study aimed to determine whether the use of thromboelastography in adult patients with cirrhosis decreases blood product use and impacts adverse events or mortality compared with standard practice. A registered (PROSPERO CRD42020192458) systematic review and meta-analysis was conducted for randomized controlled trials (RCTs) comparing thromboelastography-guided hemostatic management versus standard practice (control). Co-primary outcomes were the number of transfused platelet units and fresh frozen plasma (FFP) units. Secondary outcomes were mortality, adverse events, utilization of individual blood products, blood lo...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Hartmann, Jan Dias, Joao D. Pivalizza, Evan G. Garcia-Tsao, Guadalupe Tags: Review Article Source Type: research

Investigating a Signal of Acquired Hemophilia Associated with COVID-19 Vaccination: A Systematic Case Review
We present the result of a signal detection analysis aimed at exploring a possible association between COVID-19 immunization and occurrence of AHA. A disproportionality analysis on the World Health Organization (WHO) database was performed to investigate the presence of a signal of risk for AHA associated with COVID-19 vaccines. Reports of AHA associated with any COVID-19 vaccine included in the WHO database were then integrated with those available on the Food and Drug Administration Vaccine Adverse Events Reporting System and those published in the medical literature. The WHO database included 146 reports of AHA. The inf...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Franchini, Massimo Cappello, Emiliano Valdiserra, Giulia Bonaso, Marco Moretti, Ugo Focosi, Daniele Tuccori, Marco Tags: Review Article Source Type: research

Laboratory Diagnosis of von Willebrand Disease (VWD): Geographical Perspectives
This report reviews the laboratory aided diagnosis or exclusion of VWD from a geographic perspective, and focuses on the disparities of approaches and methods in different regions of the world. This is primarily done from the perspective of data available from published reports related to external quality assessment (or proficiency testing) from different geographic localities. Moreover, differences in approaches between laboratories may arise due to differential adherence of particular guidelines, as well as regulatory aspects and predominance of local manufacturers and suppliers. [...] Thieme Medical Publishers, Inc. 333...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Favaloro, Emmanuel J. Pasalic, Leonardo Tags: Review Article Source Type: research

One Hundred Years of Congenital Fibrinogen Disorders
Semin Thromb Hemost DOI: 10.1055/s-0042-1756187Congenital fibrinogen disorders encompass a broad range of fibrinogen defects characterized by a wide molecular and clinical spectrum. From the first clinical description of afibrinogenemia in 1920, many major achievements have contributed to a better understanding of these complex disorders. The finding of causative mutations in all three fibrinogen genes has contributed to reveal the molecular mechanisms involved in biosynthesis of the fibrinogen molecule and to clarify the basic processes of fibrin polymerization and fibrinolysis. The compilation of abundant cases with deta...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Casini, Alessandro Moerloose, Philippe de Neerman-Arbez, Marguerite Tags: Review Article Source Type: research

Evolving Knowledge on Primary and Secondary Prevention of Venous Thromboembolism in Carriers of Hereditary Thrombophilia: A Narrative Review
Semin Thromb Hemost DOI: 10.1055/s-0042-1753527The association between heritability of venous thromboembolism (VTE) and thrombophilia was first reported clinically in 1956, later followed by the first description of a congenital cause of hypercoagulability—antithrombin deficiency—in 1965. Since then, our knowledge of hereditary causes of hypercoagulability, which may predispose carriers to VTE has improved greatly. Novel genetic defects responsible for severe thrombophilia have been recently identified and we have learned that a wide range of interactions between thrombophilia and other genetic and acquired risk factor...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Campello, Elena Prandoni, Paolo Tags: Review Article Source Type: research

Activated Partial Thromboplastin Time and Prothrombin Time Mixing Studies: Current State of the Art
Semin Thromb Hemost DOI: 10.1055/s-0042-1756196Mixing studies have long been in the clinical laboratory armamentarium for investigating unexpected, prolonged activated partial thromboplastin time (aPTT) or prothrombin time (PT). The purpose of the mixing study is to identify whether the aPTT/PT prolongation is secondary to a factor deficiency versus an inhibitor, which would present as a “corrected” and “noncorrected” mixing study, respectively. The differentiation between a factor deficiency and inhibitor may likely further direct clinical decisions, including additional diagnostic testing or factor replacement th...
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Adcock, Dorothy M. Moore, Gary W. Montalv ão, Silmara de Lima Kershaw, Geoffrey Gosselin, Robert C. Tags: Review Article Source Type: research

Preface: Laboratory Diagnostics for Thrombosis and Hemostasis Testing – Part I
Semin Thromb Hemost DOI: 10.1055/s-0042-1755367 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Smock, Kristi J. Gosselin, Robert C. Tags: Preface Source Type: research

Against Therapeutic Anticoagulation in Critically Ill COVID-19 Patients
Semin Thromb Hemost DOI: 10.1055/s-0042-1756185 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Rizk, John G. Lavie, Carl J. Henry, Brandon Michael Tags: Commentary Source Type: research

Increased HDL-C Values and Mortality: Revolutionizing a Historical Paradigm?
Semin Thromb Hemost DOI: 10.1055/s-0042-1753484 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Lippi, Giuseppe Sanchis-Gomar, Fabian Lavie, Carl J. Tags: Commentary Source Type: research

Strength of Anticoagulation in Moderate to Severe COVID-19 Illness: In Medio Stat Virtus?
Semin Thromb Hemost DOI: 10.1055/s-0042-1756186 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - September 2, 2022 Category: Hematology Authors: Lippi, Giuseppe Favaloro, Emmanuel J. Tags: Commentary Source Type: research

Lupus Anticoagulant and Cardiopulmonary Bypass
Semin Thromb Hemost 2022; 48: 628-630 DOI: 10.1055/s-0042-1750045 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - July 26, 2022 Category: Hematology Authors: Arachchillage, Deepa J. Crossette-Thambiah, Christina Laffan, Mike Tags: Letter to the Editor Source Type: research

Colchicine as a Modulator of Platelet Function: A Systematic Review
Semin Thromb Hemost 2022; 48: 552-567 DOI: 10.1055/s-0042-1749660The microtubule inhibitor and anti-inflammatory agent colchicine is used to treat a range of conditions involving inflammasome activation in monocytes and neutrophils, and is now known to prevent coronary and cerebrovascular events. In vitro studies dating back more than 50 years showed a direct effect of colchicine on platelets, but as little contemporary attention has been paid to this area, we have critically reviewed the effects of colchicine on diverse aspects of platelet biology in vitro and in vivo. In this systematic review we searched Embase, Medline...
Source: Seminars in Thrombosis and Hemostasis - July 26, 2022 Category: Hematology Authors: Reddel, Caroline J. Pennings, Gabrielle J. Chen, Vivien M. Gnanenthiran, Sonali Kritharides, Leonard Tags: Review Article Source Type: research

Editorial Compilation XII
Semin Thromb Hemost 2022; 48: 497-501 DOI: 10.1055/s-0042-1750023 Thieme Medical Publishers, Inc. 333 Seventh Avenue, 18th Floor, New York, NY 10001, USAArticle in Thieme eJournals: Table of contents  |  Full text (Source: Seminars in Thrombosis and Hemostasis)
Source: Seminars in Thrombosis and Hemostasis - July 26, 2022 Category: Hematology Authors: Favaloro, Emmanuel J. Pasalic, Leonardo Lippi, Giuseppe Tags: Preface Source Type: research