Glanzmann Thrombasthenia 10 Years Later: Progress Made and Future Directions
Semin Thromb Hemost DOI: 10.1055/s-0044-1782519Glanzmann thrombasthenia (GT) is the most common inherited platelet disorder (IPD) with mucocutaneous bleeding and a failure of platelets to aggregate when stimulated. The molecular cause is insufficient or defective αIIbβ3, an integrin encoded by the ITGA2B and ITGB3 genes. On activation αIIbβ3 undergoes conformational changes and binds fibrinogen (Fg) and other proteins to join platelets in the aggregate. The application of next-generation sequencing (NGS) to patients with IPDs has accelerated genotyping for GT; progress accompanied by improved mutation curation. The eva...
Source: Seminars in Thrombosis and Hemostasis - March 18, 2024 Category: Hematology Authors: Nurden, Alan T. Nurden, Paquita Tags: Review Article Source Type: research

Prophylactic Treatment of Children with Hemophilia in Sweden
Semin Thromb Hemost DOI: 10.1055/s-0044-1782518Hemophilia A/B are caused by deficiency or lack of coagulation factors VIII (FVIII) or factor IX (FIX), respectively, in plasma. A person with hemophilia develops bleeding in the joints and muscles at an early age, which, if left untreated, leads to early arthropathy. Preventive treatment can be achieved by regular (prophylactic) administration of FVIII/FIX. In 1958, this was implemented on a small scale in Sweden with FVIII in patients with severe hemophilia A, and in those with hemophilia B in 1972 when FIX became available. However, there were problems with human immunodefi...
Source: Seminars in Thrombosis and Hemostasis - March 18, 2024 Category: Hematology Authors: Ljung, Rolf Tags: Review Article Source Type: research

Pearls and Pitfalls in the Measurement of Direct Oral Anticoagulants
Semin Thromb Hemost DOI: 10.1055/s-0044-1782196Due to their widespread use, testing for direct oral anticoagulants (DOACs) has become urgent in certain clinical situations. Screening based on widely available, rapid, and simple hemostasis assays such as prothrombin time, activated partial thromboplastin time, or even diluted Russel Viper venom time may provide sufficient evidence of “over-coagulation” and could be used “in small/peripheral/spoke laboratories” as an emergency strategy, but is not thought to be reliable for driving clinical decision making. Given their good correlation with plasma concentration, urin...
Source: Seminars in Thrombosis and Hemostasis - March 8, 2024 Category: Hematology Authors: Lippi, Giuseppe Favaloro, Emmanuel J. Tags: Review Article Source Type: research

Editorial Compilation XV
Semin Thromb Hemost DOI: 10.1055/s-0044-1782195 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 - March 8, 2024 Category: Hematology Authors: Favaloro, Emmanuel J. Pasalic, Leonardo Lippi, Giuseppe Tags: Preface Source Type: research

2024 Eberhard F. Mammen Award Announcements: Part I —Most Popular Articles
Semin Thromb Hemost DOI: 10.1055/s-0044-1782197 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 - March 8, 2024 Category: Hematology Authors: Favaloro, Emmanuel J. Tags: Editorial Source Type: research

Fibrinolytic Agents in Thromboembolic Diseases: Historical Perspectives and Approved Indications
Semin Thromb Hemost DOI: 10.1055/s-0044-1781451Fibrinolytic agents catalyze the conversion of the inactive proenzyme plasminogen into the active protease plasmin, degrading fibrin within the thrombus and recanalizing occluded vessels. The history of these medications dates to the discovery of the first fibrinolytic compound, streptokinase, from bacterial cultures in 1933. Over time, researchers identified two other plasminogen activators in human samples, namely urokinase and tissue plasminogen activator (tPA). Subsequently, tPA was cloned using recombinant DNA methods to produce alteplase. Several additional derivatives o...
Source: Seminars in Thrombosis and Hemostasis - March 1, 2024 Category: Hematology Authors: Rashedi, Sina Greason, Christie M. Sadeghipour, Parham Talasaz, Azita H. O'Donoghue, Michelle L. Jimenez, David Monreal, Manuel Anderson, Christopher D. Elkind, Mitchell S. V. Kreuziger, Lisa M. Baumann Lang, Irene M. Goldhaber, Samuel Z. Konstantinides, Tags: Review Article Source Type: research

Variable Performance of D-dimer Testing by Hemostasis Laboratories: The Australasian/Asia-Pacific Experience
Semin Thromb Hemost DOI: 10.1055/s-0044-1781450D-dimers represent the breakdown products of fibrin. Thus, elevated plasma D-dimers will arise following a thrombotic event, such as a deep vein thrombosis or a pulmonary embolism, and therefore, a nonelevated D-dimer is used to effectively exclude such events. D-dimers are also elevated in a range of other conditions, for example, during disseminated intravascular coagulation. D-dimer levels may also be associated with prognostic value. For example, highly raised D-dimer levels can be associated with worsening clinical features in coronavirus disease 2019. Thus, D-dimer testi...
Source: Seminars in Thrombosis and Hemostasis - March 1, 2024 Category: Hematology Authors: Favaloro, Emmanuel J. Arunachalam, Sandya Dean, Elysse Tags: Review Article Source Type: research

Lupus Anticoagulant-Hypoprothrombinemia Syndrome: Literature Review and Description of Local Case in a 3-Year-Old Chinese Girl
In conclusion, LAHPS is most commonly observed in female patients, particularly those under 18 years of age. LAHPS is characterized by hemorrhage, occurring at various sites and with varying degrees of severity, but the majority of patients improve with appropriate treatment and management. [...] 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 - February 23, 2024 Category: Hematology Authors: Wang, Bin Tang, Ning Zhang, Chi Tags: Review Article Source Type: research

Venous Thrombosis in Airborne Viral Infections: Is Coronavirus Disease 2019 now Any Different from Influenza?
Semin Thromb Hemost DOI: 10.1055/s-0044-1780507One of the hallmarks of coronavirus disease 2019 (COVID-19), particularly in complicated cases (i.e., requiring hospitalization or intensive care support), is persistent hemostasis activation, which may be associated with a vast array of thrombotic episodes involving both the arterial and venous systems. The renewed emphasis on the relationship between viral infections and venous thrombosis paves the way for determining whether a more common and often underestimated infection disease, such as influenza, may also be associated with a significant burden of venous thrombotic epis...
Source: Seminars in Thrombosis and Hemostasis - February 23, 2024 Category: Hematology Authors: Lippi, Giuseppe Favaloro, Emmanuel J. Nocini, Riccardo Tags: Review Article Source Type: research

Innovative Therapies for Acquired Hemophilia A
Semin Thromb Hemost DOI: 10.1055/s-0044-1779737Acquired hemophilia A (AHA) is a rare autoimmune bleeding disorder which can be life-threatening. AHA is due to autoantibodies against coagulation factor VIII. Disease onset may be idiopathic (approximately half of the cases) or triggered by autoimmune disorders, cancers, drugs, infections, or pregnancy. Besides treating the underlying disorder, specific AHA treatments include management of bleeding and inhibitor eradication. Various first-line and second-line hemostatic and immunosuppressive agents are currently available for the management of AHA. Recently, the hemostatic dr...
Source: Seminars in Thrombosis and Hemostasis - February 23, 2024 Category: Hematology Authors: Franchini, Massimo Focosi, Daniele Tags: Review Article Source Type: research

Fibrinolysis-Mediated Pathways in Acute Liver Injury
Semin Thromb Hemost DOI: 10.1055/s-0044-1779738Acute liver injury (ALI), that is, the development of reduced liver function in patients without preexisting liver disease, can result from a wide range of causes, such as viral or bacterial infection, autoimmune disease, or adverse reaction to prescription and over-the-counter medications. ALI patients present with a complex coagulopathy, characterized by both hypercoagulable and hypocoagulable features. Similarly, ALI patients display a profound dysregulation of the fibrinolytic system with the vast majority of patients presenting with a hypofibrinolytic phenotype. Decades o...
Source: Seminars in Thrombosis and Hemostasis - February 23, 2024 Category: Hematology Authors: Capece, Gina E. Luyendyk, James P. Poole, Lauren G. Tags: Review Article Source Type: research

Risk of Venous Thromboembolism by Cancer Type: A Network Meta-Analysis
This study was conducted to estimate the risk of VTE by cancer type and factors influencing VTE risk. The Embase, MEDLINE, and Cochrane Library repositories were systematically searched to identify clinical trials and observational studies published from 2005 to 2022 that assessed the risk of primary cancer-related VTE among two or more distinct cancer types. Studies with similar cancer populations and study methods reporting VTE occurring within 1 year of diagnosis were included in the NMA. Relative VTE rates across cancer types were estimated with random-effects Bayesian NMAs. Absolute VTE rates were calculated from thes...
Source: Seminars in Thrombosis and Hemostasis - February 23, 2024 Category: Hematology Authors: Betts, Marissa B. Liu, Xuejun Junqueira, Daniela R. Fahrbach, Kyle Neupane, Binod Ronnebaum, Sarah Dhamane, Amol D. Tags: Review Article Source Type: research

Development of a Coagulation Disorders Unit
Semin Thromb Hemost DOI: 10.1055/s-0044-1779633Our Coagulation Disorders Unit in Helsinki, Finland, provides 24/7 services for local and national hospitals and colleagues upon requests regarding bleeding and thrombosis diagnostics and management, including follow-up. The unit has a tight connection between the clinic and laboratory, and its maintenance and sharing knowledge and observations have been priorities, already for over 20 years and will continue to be of major importance. The consultation service is provided by phone during daytime and on-call hours, and in written form sent electronically to the consulting stake...
Source: Seminars in Thrombosis and Hemostasis - February 19, 2024 Category: Hematology Authors: Szanto, Timea Helin, Tuukka Joutsi-Korhonen, Lotta Lehtinen, Anna-Elina El Beayni, Nancy Lep äntalo, Aino Lassila, Riitta Tags: Review Article Source Type: research

The History of Diagnosing Venous Thromboembolism
Semin Thromb Hemost DOI: 10.1055/s-0044-1779484An accurate and prompt diagnosis of deep vein thrombosis and/or pulmonary embolism is important to prevent serious complications and mortality. Because the clinical presentation of venous thromboembolism (VTE) is often nonspecific, objective testing by means of radiological imaging is required to confirm the diagnosis. Historically, a diagnosis of VTE involved invasive imaging techniques like contrast venography or conventional pulmonary angiography. Technological developments toward more accurate and less invasive diagnostics have driven the implementation of a variety of new...
Source: Seminars in Thrombosis and Hemostasis - February 19, 2024 Category: Hematology Authors: Martens, Emily S.L. Huisman, Menno V. van Mens, Thijs E. Klok, Frederikus A. Tags: Review Article Source Type: research

Tranexamic Acid: An Evergreen Hemostatic Agent
Semin Thromb Hemost DOI: 10.1055/s-0044-1779632Tranexamic acid (TXA) is an important antifibrinolytic agent, which inhibits plasminogen activation and fibrinolysis. Several controlled randomized trials have investigated the role of TXA in preventing or decreasing blood loss across different surgical interventions or medical conditions characterized by excessive bleeding, consistently documenting its effectiveness and safety. Although the first clinical use of TXA dates back to more than 60 years ago, TXA remains the focus of intense research. This narrative review summarizes the more recent results and indications on the c...
Source: Seminars in Thrombosis and Hemostasis - February 9, 2024 Category: Hematology Authors: Franchini, Massimo Focosi, Daniele Mannucci, Pier Mannuccio Tags: Review Article Source Type: research