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The Current Evidence of Pulmonary Embolism Response Teams and Their Role in Future Hämostaseologie (IF 3.2) Pub Date : 2024-03-12 Lukas Hobohm, Ioannis T. Farmakis, Daniel Duerschmied, Karsten Keller
Acute pulmonary embolism (PE) remains a critical medical condition requiring prompt and accurate management. The introduction and growing significance of pulmonary embolism response teams (PERT), also termed EXPERT-PE teams, signify a paradigm shift toward a collaborative, multidisciplinary approach in managing this complex entity. As the understanding of acute PE continues to evolve, PERTs stand as
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Thrombosis in Acute Promyelocytic Leukemia: The Current Understanding Hämostaseologie (IF 3.2) Pub Date : 2024-03-11
Despite enormous improvement in the management of patients with acute promyelocytic leukemia (APL), the distinctive coagulopathy observed at presentation in affected patients is often life-threatening. While hemorrhagic manifestations are well known and described in this setting, APL-related thromboses are underappreciated. Data regarding this complication are scarce showing variable incidence. Furthermore
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Direct Oral Anticoagulants for Pulmonary Embolism Hämostaseologie (IF 3.2) Pub Date : 2024-03-11 Roberto Pizzi, Ludovica Anna Cimini, Walter Ageno, Cecilia Becattini
Venous thromboembolism (VTE) is the third most common cardiovascular disease. For most patients, the standard of treatment has long consisted on low-molecular-weight heparin followed by vitamin K antagonists, but a number of clinical trials and, subsequently, post-marketing studies have shown that direct oral anticoagulants (DOACs) with or without lead-in heparin therapy are effective alternatives
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Quantifying Residual Rivaroxaban Plasma Concentration after Antagonization with Andexanet Alfa: A Difficult Task in Routine Clinical Practice Hämostaseologie (IF 3.2) Pub Date : 2024-03-11 Alexander Mair, Gilles Huber, Jan-Dirk Studt, Donat R. Spahn, Alexander Kaserer
We describe the case of a 38-year-old man with a history of chronic portal vein thrombosis who presented with abdominal pain after a transjugular intrahepatic portosystemic shunt procedure. Under anticoagulation therapy with rivaroxaban, he experienced active splenic bleeding, leading to hemodynamic instability. Emergency interventions, including andexanet alfa and nanoparticle administration, successfully
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Type 2 Diabetes: Platelets and Long-Term Metabolic Control as Estimated from Glycosylated Haemoglobin (HbA1c) Hämostaseologie (IF 3.2) Pub Date : 2024-03-06 M. Edvardsson, M. Oweling, P. Järemo
In type 2 diabetes, platelets are likely affected by impaired long-term glycaemic control, but such pathophysiological links are poorly understood. This study thus compares platelet reactivity (i.e. agonist-evoked platelet reactions) in vitro with glycosylated haemoglobin (HbA1c), a measure commonly used for monitoring long-term metabolic control of type 2 diabetes. Elders with type 2 diabetes (n = 35)
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Assessment of Factor VIII Activity and D-Dimer Levels in the Post-COVID Period Hämostaseologie (IF 3.2) Pub Date : 2024-03-01 Mirjana Kovac, Milena Todorovic Balint, Marija Milenkovic, Dusica Basaric, Branko Tomic, Bela Balint, Vera Ignjatovic
Changes in the hemostatic system during COVID infection lead to hypercoagulability. Numerous studies have evaluated hemostatic abnormalities in COVID patients during acute infection, in the period of hospitalization. However, the hemostatic status following hospital discharge has not been sufficiently assessed. Considering the importance of FVIII and D-dimer levels as markers for the assessment of
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Anti-Thrombin IgA in a Patient with Multiple Myeloma Leading to In Vitro Interference in Multiple Coagulation Tests and Confounding Diagnosis Hämostaseologie (IF 3.2) Pub Date : 2024-03-01 Christian Irsara, Andrea Griesmacher, Lorin Loacker, Clemens Feistritzer, Cosima Anna Überbacher, Jean Amiral
Wir berichten über eine 59-jährige Myelom-Patientin, die einen IgA Autoantikörper gegen humanes Thrombin entwickelt hat. Dieser führte in vitro zu verlängerten Gerinnungszeiten und täuschte Hemmkörper gegen sämtliche intrinsische Gerinnungs-Einzelfaktoren vor, wobei die Patientin keine spontane Blutungsneigung aufwies. Der Autoantikörper beeinflusste dabei alle Gerinnungstests, in welchen humanes (nicht
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100 Years of Thrombotic Thrombocytopenic Purpura: A Story of Death and Life Hämostaseologie (IF 3.2) Pub Date : 2024-02-28
One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli Moschcowitz. In honor of this report, this article, written by distinguished specialists in TTP, reviews the increase in scientific knowledge on this disease during the last 100 years. It covers the scientific progress from plasma therapy
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Pathophysiology of Trauma-Induced Coagulopathy Hämostaseologie (IF 3.2) Pub Date : 2024-02-28 Herbert Schöchl, Felix C.F. Schmitt, Marc Maegele
Trauma-induced coagulopathy (TIC) is a complex hemostatic disturbance that can develop early after a major injury. There is no universally accepted definition of TIC. However, TIC primarily refers to the inability to achieve sufficient hemostasis in severely injured trauma patients, resulting in diffuse microvascular and life-threatening bleeding. Endogenous TIC is driven by the combination of hypovolemic
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The Concept of Thromboinflammation Hämostaseologie (IF 3.2) Pub Date : 2024-02-28
Inflammation and thrombosis are intricate and closely interconnected biological processes that are not yet fully understood and lack effective targeted therapeutic approaches. Thrombosis initiated by inflammatory responses, known as immunothrombosis, can confer advantages to the host by constraining the spread of pathogens within the bloodstream. Conversely, platelets and the coagulation cascade can
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Clonal Hematopoiesis and Cardiovascular Risk: Atherosclerosis, Thrombosis, and beyond Hämostaseologie (IF 3.2) Pub Date : 2024-02-28 Benedetta Izzi, José J. Fuster
Acquired mutations that lead to clonal hematopoiesis have emerged as a new and potent risk factor for atherosclerotic cardiovascular disease and other cardiovascular conditions. Human sequencing studies and experiments in mouse models provide compelling evidence supporting that this condition, particularly when driven by specific mutated genes, contributes to the development of atherosclerosis by exacerbating
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PAS: Die Rolle von Natriumcitrat bei längerer Kaltlagerung von Thrombozyten Hämostaseologie (IF 3.2) Pub Date : 2024-02-28
Kalt gelagerte Blutplättchen finden in der Behandlung von Blutungen zunehmend Verwendung. Unterschiede in den Herstellungsverfahren und Lagerungslösungen können die Qualität der Thrombozyten und ihre Haltbarkeit jedoch beeinflussen. PAS-E und PAS-F sind in Europa und Australien bzw. in den Vereinigten Staaten zugelassene Additivlösungen (PAS). L. Johnson und Kollegen prüften nun, ob Labor- und klinische
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Thrombozytopenie: Sind Thrombozytentransfusionen vor Legen eines ZVK verzichtbar? Hämostaseologie (IF 3.2) Pub Date : 2024-02-28
Die Transfusionsrichtlinien bezüglich der Schwellenwerte für die Thrombozytenzahl vor dem Legen eines zentralen Venenkatheters (ZVK) enthalten widersprüchliche Empfehlungen, da qualitativ hochwertige und aussagekräftige Belege fehlen. Der routinemäßige Einsatz von Ultraschall hat die Blutungskomplikationen verringert. F. L. F. van Baarle und Kollegen überprüften nun, wie der Verzicht auf entsprechende
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Vakzin-induzierte Immunthrombozytopenie und Thrombose: Langfristiger Outcome Hämostaseologie (IF 3.2) Pub Date : 2024-02-28
Die rasche Diagnose und Behandlung hat den Outcome von Patienten mit vakzin-induzierter Immunthrombozytopenie und -thrombose (VITT) verbessert. Nach der akuten Episode sind jedoch viele Fragen hinsichtlich des langfristigen Managements einer VITT ungeklärt. Diesen widmeten sich nun L. Schönborn und Kollegen.
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Bleeding Disorder of Unknown Cause: A Diagnosis of Exclusion Hämostaseologie (IF 3.2) Pub Date : 2024-02-27 Dino Mehic, Johanna Gebhart, Ingrid Pabinger
Patients with an unexplained mild to moderate bleeding tendency are diagnosed with bleeding disorder of unknown cause (BDUC), a classification reached after ruling out other mild to moderate bleeding disorders (MBD) including von Willebrand disease (VWD), platelet function defects (PFDs), coagulation factor deficiencies (CFDs), and non-hemostatic causes for bleeding. This review outlines our diagnostic
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VTE Risk Assessment and Prevention in Pregnancy Hämostaseologie (IF 3.2) Pub Date : 2024-02-26 Ellen O'Rourke, Rehman Faryal, Marc Blondon, Saskia Middeldorp, Fionnuala Ní Áinle
Venous thromboembolism (VTE) remains the leading cause of maternal mortality in pregnancy and the postpartum period. In addition to the higher pregnancy-associated baseline VTE risk, there are several well-established risk factors that can further increase the risk of VTE. At present, a thorough interrogation of these risk factors remains our only tool for estimating which pregnant people may be at
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Inflammation, Sepsis, and the Coagulation System Hämostaseologie (IF 3.2) Pub Date : 2024-02-14 János Kappelmayer, Ildikó Beke Debreceni, Zsolt Fejes, Béla Nagy Jr.
Sepsis has been a major health problem for centuries and it is still the leading cause of hospital deaths. Several studies in the past decades have identified numerous biochemical abnormalities in severe cases, and many of these studies provide evidence of the perturbation of the hemostatic system. This can result in complications, such as disseminated intravascular coagulation that can lead to multiorgan
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Splanchnic Vein Thrombosis: The State-of-the-Art on Anticoagulant Treatment Hämostaseologie (IF 3.2) Pub Date : 2024-02-14
Splanchnic vein thrombosis (SVT) is a rare type of venous thromboembolism occurring within the splanchnic venous system. Portal vein thrombosis is the most common presentation, while Budd–Chiari syndrome is the least common. Liver cirrhosis and abdominal solid cancer are the main local risk factors for SVT, whereas myeloproliferative neoplasms are the predominant systemic risk factors. Signs and symptoms
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Multisite Thrombosis in a Patient with Paroxysmal Nocturnal Hemoglobinuria Hämostaseologie (IF 3.2) Pub Date : 2024-02-09 Lennart Beckmann, Tobias D. Faizy, Fabian Flottmann, Jens Fiehler, Carsten Bokemeyer, Lennart Well, Antonia Beitzen-Heineke, Florian Langer
Case: Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare bone marrow disorder caused by acquired mutations in the phosphatidylinositol glycan class A gene, which lead to a partial or total loss of the cellular complement regulators CD55 and CD59.1 In addition to complement-mediated hemolysis and cytopenia, venous and arterial thromboses at multiple and/or unusual sites are a common complication
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Oral Anticoagulation and Mortality in Cases with Intracranial Bleeding: Analysis of Nationwide Prescription and Hospitalization Data Hämostaseologie (IF 3.2) Pub Date : 2024-02-05 Knut Kröger, Fabian Heldt, Ludger Feyen, Kathrin Feller, Bernd Kowall, Andreas Stang
Objectives To demonstrate the safety of direct oral anticoagulants in relation to intracranial bleeding (ICB), we compared the number of patients taking anticoagulants in all cases of hospitalization and cases of hospitalization for ICB over time in Germany. We analyzed the intrahospital mortality of ICB cases in relation to long-term use of anticoagulants (LUAs). We performed a retrospective registry
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Update on Thrombosis Risk in Patients with Cancer: Focus on Novel Anticancer Immunotherapies Hämostaseologie (IF 3.2) Pub Date : 2024-01-08 Florian Moik, Jakob M. Riedl, Cornelia Englisch, Cihan Ay
Thromboembolic complications, including venous thromboembolism (VTE) and arterial thromboembolism (ATE), increase mortality and morbidity, and delay treatment in patients with cancer. Therefore, an increased understanding of underlying risk profiles, the identification of risk factors and predictive biomarkers, and ultimately the development of specific cardiovascular prevention strategies in patients
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Programm für die Veranstaltung des BDDH im Rahmen der 68. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung in Wien 2024 und Petition an den Deutschen Bundestag aufgrund eines drohenden Praxenkollaps Hämostaseologie (IF 3.2) Pub Date : 2023-12-14 Jürgen Koscielny, Günther Kappert, Christoph Sucker
Veranstaltung des BDDH auf dem 68. GTH-Jahreskongress 2024 Vom 27.02. – 01.03.2024 findet der 68. Jahreskongress der Gesellschaft für Thrombose- und Hämostaseforschung in Wien statt. Im Rahmen des Kongresses findet auch eine Sitzung des Berufsverbandes der Deutschen Hämostaseologen (BDDH) statt. Den Vorsitz übernehmen Jürgen Koscielny als 1. Vorsitzender und Günther Kappert als 2. Vorsitzender des
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Marker der Akute-Phase-Reaktion könnten Blutungen bei Hämophilie detektieren Hämostaseologie (IF 3.2) Pub Date : 2023-12-14
Bei der Hämophilie A und B werden im Rahmen von akuten Hämorrhagien Entzündungsreaktionen ausgelöst. Sind Gelenke von den Blutungen betroffen, kann hieraus die so genannte hämophile Arthropathie resultieren. In einer aktuellen Studie wurden jetzt Patienten mit Hämophilie im Hinblick auf Marker der Akute-Phase-Reaktion untersucht. Darüber hinaus wurde ein Tiermodell etabliert.
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Potenzielle globale Gerinnungsparameter für Therapiemonitoring unter Emicizumab Hämostaseologie (IF 3.2) Pub Date : 2023-12-14
Während die Behandlung der genetisch bedingten Gerinnungsstörung Hämophilie A in der Vergangenheit in regelmäßigen intravenösen Faktor-VIII-Infusionen bestand, steht heute mit dem Antikörper Emicizumab eine effektive Alternative zur subkutanen Applikation zur Verfügung. Bisher fehlen allerdings Parameter, mit denen sich das Ausmaß der Gerinnungskorrektur beim einzelnen Patienten verlässlich abbilden
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Classic Light Transmission Platelet Aggregometry: Do We Still Need it? Hämostaseologie (IF 3.2) Pub Date : 2023-12-08 Jennifer Gebetsberger, Florian Prüller
For more than 50 years, light transmission aggregometry has been accepted as the gold standard test for diagnosing inherited platelet disorders in platelet-rich plasma, although there are other functional approaches performed in whole blood. In this article, several advantages and disadvantages of this technique over other laboratory approaches are discussed in the view of recent guidelines, and the
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Emicizumab for the Treatment of Acquired Hemophilia A: Consensus Recommendations from the GTH-AHA Working Group Hämostaseologie (IF 3.2) Pub Date : 2023-12-04 Christian Pfrepper, Robert Klamroth, Johannes Oldenburg, Katharina Holstein, Hermann Eichler, Christina Hart, Patrick Moehnle, Kristina Schilling, Karolin Trautmann-Grill, Mohammed Alrifai, Cihan Ay, Wolfgang Miesbach, Paul Knoebl, Andreas Tiede
Background Acquired hemophilia A (AHA) is a severe bleeding disorder caused by autoantibodies against coagulation factor VIII (FVIII). Standard treatment consists of bleeding control with bypassing agents and immunosuppressive therapy. Emicizumab is a bispecific antibody that mimics the function of activated FVIII irrespective of the presence of neutralizing antibodies. Recently, the GTH-AHA-EMI study
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Cancer-Associated Venous Thromboembolism—Diagnostic and Therapeutic Considerations: An Update Based on the Revised AWMF S2k Guideline Hämostaseologie (IF 3.2) Pub Date : 2023-11-22
Patients with cancer are prone to develop venous thromboembolism (VTE) with negative impact on quality of life, morbidity, and mortality. Treatment of established VTE is often complex in patients with cancer. Treatment of cancer-associated VTE (CAT) basically comprises initial and maintenance treatment, for 3 to 6 months, secondary preventions, and treatment in special situations. Therapeutic anticoagulation
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Diagnosis and Therapy of Visceral Vein Thrombosis: An Update Based on the Revised AWMF S2k Guideline Hämostaseologie (IF 3.2) Pub Date : 2023-11-22 Katja S. Mühlberg
Splanchnic or visceral vein thromboses (VVTs) are atypical thrombotic entities and include thrombosis of the portal vein, hepatic veins (Budd-Chiari syndrome), mesenteric veins, and splenic vein. All VVTs have in common high 30-day mortality up to 20% and it seems to be difficult to diagnose VVT early because of their rarity and their wide spectrum of unspecific symptoms. VVTs are often associated
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THROMKIDplus Patient Registry and Biomaterial Banking for Children with Inherited Platelet Disorders Hämostaseologie (IF 3.2) Pub Date : 2023-11-02 Matthias Ballmaier, Manuela Germeshausen, Harald Schulze, Oliver Andres, on behalf of the THROMKIDplus Study Group
Inherited platelet disorders (IPDs) represent a heterogeneous group of disorders that include both quantitative (thrombocytopenia or thrombocytosis) and qualitative (thrombocytopathy) defects. To gain better knowledge about the prevalence, pathogenesis, and clinical consequences of specific diseases, to improve diagnosis and treatment of patients with IPD, and to support translational research on a
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Management of Recurrent Venous Thromboembolism in Severe Immune Thrombocytopenia: A Case Report and a Review of the Literature Hämostaseologie (IF 3.2) Pub Date : 2023-10-31
We report a case of a 58-year-old man with recurrent unprovoked deep vein thrombosis (DVT) and severe immune thrombocytopenia (ITP) with a platelet count of 19 × 109/L. We further review studies reporting venous thromboembolism (VTE) in patients with severe ITP (≤ 35 × 109/L) and identified 14 patients highlighting VTE risk factors and management of these patients. The present case had several risk
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Preexisting Chronic Thromboembolic Pulmonary Hypertension in Acute Pulmonary Embolism? A Case Report and Discussion Hämostaseologie (IF 3.2) Pub Date : 2023-10-30
A 61-year-old male presented with New York Heart Association class II breathlessness. Three years earlier, he had presented with a swollen leg, had received a diagnosis of deep vein thrombosis on ultrasound and of low-risk acute pulmonary embolism, and had been discharged on a direct oral anticoagulant after 8 hours. The patient also had a history of thyroidectomy and was on levothyroxine substitution
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Subsegmental Pulmonary Embolism Hämostaseologie (IF 3.2) Pub Date : 2023-10-23 Christine Baumgartner, Tobias Tritschler, Drahomir Aujesky
Subsegmental pulmonary embolism (SSPE) is increasingly diagnosed with the growing use and technological advancements of multidetector computed tomography pulmonary angiography. Its diagnosis is challenging, and some presumed SSPE may actually represent imaging artifacts. Indirect evidence and results from small observational studies suggest that SSPE may be more benign than more proximal pulmonary
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Intravital Imaging of Thrombosis Models in Mice Hämostaseologie (IF 3.2) Pub Date : 2023-10-19 Klytaimnistra Kiouptsi, Martina Casari, Jonathan Mandel, Zhenling Gao, Carsten Deppermann
Intravital microscopy is a powerful tool to study thrombosis in real time. The kinetics of thrombus formation and progression in vivo is studied after inflicting damage to the endothelium through mechanical, chemical, or laser injury. Mouse models of atherosclerosis are also used to induce thrombus formation. Vessels of different sizes and from different vascular beds such as carotid artery or vena
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Flow Chamber Analyses in Cardiovascular Research: Impact of Platelets and the Intercellular Crosstalk with Endothelial Cells, Leukocytes, and Red Blood Cells Hämostaseologie (IF 3.2) Pub Date : 2023-10-19 Kim Jürgen Krott, Tobias Feige, Margitta Elvers
Platelets are main drivers of thrombus formation. Besides platelet aggregate formation, platelets interact with different blood cells such as red blood and white blood cells (RBCs, WBCs) and endothelial cells (ECs), to promote thrombus formation and inflammation. In the past, the role of different proteins in platelet adhesion, activation, and aggregate formation has been analyzed using platelets/mice
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Potentials of Endothelial Colony-Forming Cells: Applications in Hemostasis and Thrombosis Disorders, from Unveiling Disease Pathophysiology to Cell Therapy Hämostaseologie (IF 3.2) Pub Date : 2023-10-19 Nadine Schwarz, Hamideh Yadegari
Endothelial colony-forming cells (ECFCs) are endothelial progenitor cells circulating in a limited number in peripheral blood. They can give rise to mature endothelial cells (ECs) and, with intrinsically high proliferative potency, contribute to forming new blood vessels and restoring the damaged endothelium in vivo. ECFCs can be isolated from peripheral blood or umbilical cord and cultured to generate
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Geringe α-Thrombin/GPIbα-Interaktion trägt möglicherweise zur Hyperreaktivität der Thrombozyten bei COVID-19-Patienten bei Hämostaseologie (IF 3.2) Pub Date : 2023-10-19
Mehrere Studien haben gezeigt, dass Thrombozyten mit dem SARSCoV-2 Coronavirus interagieren können. In der Folge kommt es zum programmierten Zelltod, zur Freisetzung extrazellulärer Vesikel und zu einer erhöhten Thrombozytenreaktivität bei Patienten mit der Coronavirus-Krankheit-2019 (COVID-19) als Reaktion auf niedrige Dosen von α-Thrombin.
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Leichte Schlaganfälle: Thrombolyse und routinemäßige medi-zinische Versorgung im Vergleich Hämostaseologie (IF 3.2) Pub Date : 2023-10-19
Ungefähr 30% der jährlich neu auftretenden Schlaganfälle in China werden als leichte ischämische Schlaganfälle eingestuft. Fast ein Drittel dieser Patienten sind drei Monate nach dem Indexereignis entweder schwer beeinträchtigt oder verstorben. Obwohl es keinen klaren Standard für die Verabreichung einer thrombolytischen Therapie innerhalb des 4,5-Stunden-Fensters gibt, wurde in den letzten Jahren
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Animal and Cellular Models in Thrombosis and Hemostasis Hämostaseologie (IF 3.2) Pub Date : 2023-10-19 Christoph Reinhardt, Heiko Rühl
Standardized In Vitro and In Vivo Model Systems to Simplify Complexity—That's How We Learn The discovery of new target molecules and translational progress in the development and refinement of antithrombotic therapies as well as the improved treatment of bleeding disorders strongly relies on standardized ex vivo and in vivo models that closely resemble the respective human pathologies. The standardization
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Grundlagen zur Anwendung von Andexanet Hämostaseologie (IF 3.2) Pub Date : 2023-10-09 J. Koscielny, I. Birschmann, R. Bauersachs, D. Trenk, F. Langer, P. Möhnle, J. Beyer-Westendorf
Hintergrund Für lebensbedrohliche oder unkontrollierbare Blutungen steht bei einer Assoziation mit dem Thrombin-Inhibitor Dabigatran das monoklonale Antikörperfragment Idarucizumab und bei einer Assoziation mit den direkten Faktor-Xa-Inhibitoren Rivaroxaban oder Apixaban das modifizierte rekombinante FXa-Protein Andexanet für eine Antagonisierung zur Verfügung. Diese spezifischen Antidote stellen Notfallpräparate
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Arterial and Venous Thromboembolic Complications in 832 Patients with BCR-ABL-Negative Myeloproliferative Neoplasms Hämostaseologie (IF 3.2) Pub Date : 2023-10-09 Kai Wille, Eva Deventer, Parvis Sadjadian, Tatjana Becker, Vera Kolatzki, Karlo Hünerbein, Raphael Meixner, Marina Jiménez-Muñoz, Christiane Fuchs, Martin Griesshammer
Arterial (ATE) and venous (VTE) thromboembolic complications are common causes of morbidity and mortality in BCR-ABL-negative myeloproliferative neoplasms (MPNs). However, there are few studies that include all MPN subtypes and focus on both MPN-associated ATE and VTE. In our single-center retrospective study of 832 MPN patients, a total of 180 first thromboembolic events occurred during a median follow-up
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Clinical Characterization and Molecular Analysis of Fourteen Chinese Patients with Factor V Deficiency Hämostaseologie (IF 3.2) Pub Date : 2023-09-15 Ke Zhang, Longying Ye, Yanhui Jin, Yuan Chen, Manlin Zeng, Kaiqi Jia, Lihong Yang, Mingshan Wang
Introduction Coagulation factor V (FV) functions as a vital cofactor that performs procoagulant roles in the coagulation system. We investigated 14 unrelated patients whose plasma FV levels were all below the reference range. Methods FV activity (FV:C) and FV antigen were detected by one-stage clotting and ELISA, respectively. All 25 exons of the F5 gene in patients were amplified by the PCR, and they
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The Use of Large Animal Models in Trauma and Bleeding Studies Hämostaseologie (IF 3.2) Pub Date : 2023-09-11 Farahnaz Rayatdoost, Oliver Grottke
Background Major trauma often results in significant bleeding and coagulopathy, posing a substantial clinical burden. To understand the underlying pathophysiology and to refine clinical strategies to overcome coagulopathy, preclinical large animal models are often used. This review scrutinizes the clinical relevance of large animal models in hemostasis research, emphasizing challenges in translating
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Nachtrag zur kritischen Diskussion über das Deutsche Hämophilieregister (DHR) anlässlich der GTH-Jahrestagung in Frankfurt 2023, aktuelle Informationen zur Gefährdung der Versorgung mit Blutplasmaprodukten und Aus für Siemens Atellica in Deutschland Hämostaseologie (IF 3.2) Pub Date : 2023-08-31 Jürgen Koscielny, Günther Kappert, Christoph Sucker
Nachtrag zur kritischen Diskussion über das Deutsche Hämophilieregister (DHR) anlässlich der GTH-Jahrestagung in Frankfurt 2023 Mittlerweile sind drei Monate seit der BDDH-Veranstaltung im Rahmen der GTH-Jahrestagung in Frankfurt vergangen. Mehrere Referenten hatten hier konstruktiv-kritische Kritik an der aktuellen Gestaltung und Datenabfrage des Deutschen Hämophilieregisters (DHR) geübt. Die Kritik
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Treatment of Inherited Platelet Disorders: Current Status and Future Options Hämostaseologie (IF 3.2) Pub Date : 2023-08-23 Caroline Bargehr, Ralf Knöfler, Werner Streif
Inherited platelet disorders (IPDs) comprise a heterogeneous group of entities that manifest with variable bleeding tendencies. For successful treatment, the underlying platelet disorder, bleeding severity and location, age, and sex must be considered in the broader clinical context. Previous information from the AWMF S2K guideline #086–004 (www.awmf.org) is evaluated for validity and supplemented
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Thrombocytopenia Absent Radius (TAR)-Syndrome: From Current Genetics to Patient Self-Empowerment Hämostaseologie (IF 3.2) Pub Date : 2023-08-23 Gabriele Strauss, Kristina Mott, Eva Klopocki, Harald Schulze
Thrombocytopenia absent radius (TAR) syndrome is a rare form of hereditary thrombocytopenia associated with a bilateral radial aplasia. TAR syndrome is genetically defined by the combination of a microdeletion on chromosome 1 which includes the gene RBM8A, and a single nucleotide polymorphism (SNP) in the second RBM8A allele. While most patients with TAR syndrome harbor a SNP in either the 5′ UTR region
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State-of-the-Art Targeted High-Throughput Sequencing for Detecting Inherited Platelet Disorders Hämostaseologie (IF 3.2) Pub Date : 2023-08-23 Jennifer Gebetsberger, Kristina Mott, Aline Bernar, Eva Klopocki, Werner Streif, Harald Schulze
Inherited platelet disorders (IPDs) are a heterogeneous group of rare entities caused by molecular divergence in genes relevant for platelet formation and function. A rational diagnostic approach is necessary to counsel and treat patients with IPDs. With the introduction of high-throughput sequencing at the beginning of this millennium, a more accurate diagnosis of IPDs has become available. We discuss
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Postthrombotisches Syndrom: SOX-PTS-Score und Méan-Modell zeigen beste Prädiktion Hämostaseologie (IF 3.2) Pub Date : 2023-08-23
Das postthrombotische Syndrom stellt die häufigste Komplikation einer tiefen Venenthrombose dar: 20-50% der Patienten mit proximaler tiefer Venenthrombose der unteren Extremität sind davon betroffen. Von drei verschiedenen etablierten Risikoprädiktionsmodellen für die Manifestation eines postthrombotischen Syndroms wiesen zwei eine gute Prädiktion auf.
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Erhöhte Faktor-VIII-Aktivität – gesteigertes Risiko für Pfortaderthrombosen Hämostaseologie (IF 3.2) Pub Date : 2023-08-23
Pfortaderthrombosen kommen bei Patienten mit Leberzirrhose häufig vor. Sie können, auch wenn sie häufig als Zufallsbefund diagnostiziert werden, eine portale Hypertension aggravieren und das Risiko für gastrointestinale Varizenblutungen erhöhen. Eine aktuelle Studie zeigte, dass eine erhöhte Faktor-VIII-Aktivität mit einer erhöhten Inzidenz und Schwere von Pfortaderthrombosen einhergehen könnte.
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Progress in Hemostasis (Part 1): Improved Management of Inherited Platelet Disorders: Reality or Illusion? Hämostaseologie (IF 3.2) Pub Date : 2023-08-23 Werner Streif
Platelets are key drivers of hemostasis. Low platelet counts, dysfunction in platelet adhesion, and aggregation lead to increased bleeding tendency. Inherited platelet disorders (IPDs) form a highly heterogeneous group of rare diseases with variable bleeding tendency. IPDs may be associated with other signs and symptoms often referred to as “syndromic.” The underlying genetic defect may prone patients
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A Novel Fibrinogen Mutation p.BßAla68Asp Causes an Inherited Dysfibrinogenemia Hämostaseologie (IF 3.2) Pub Date : 2023-07-29 Kaiqi Jia, Manlin Zeng, Xiaoyong Zheng, Haixiao Xie, Lihong Yang, Yaosheng Xie, Mingshan Wang
Objective Our study aimed to analyze the phenotype and genotype of a pedigree with inherited dysfibrinogenemia, and preliminarily elucidate the probable pathogenesis. Methods The one-stage clotting method was used to test the fibrinogen activity (FIB:C), whereas immunoturbidimetry was performed to quantify the fibrinogen antigen (FIB:Ag). Furthermore, DNA sequence analysis was conducted to confirm
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Fibrinogen Bonn (p. Arg510Cys) in the Aα-Chain Is Associated with High Risk of Venous Thrombosis Hämostaseologie (IF 3.2) Pub Date : 2023-07-13 V. Ivaškevičius, A. Biswas, S. Singh, U. Stulpinaitė, S. Reda, H. Rühl, B. Pezeshkpoor, A. Pavlova, J. Oldenburg
Introduction Inherited dysfibrinogenemia is a qualitative defect of fibrinogen caused by various mutations among three fibrinogen genes. Dysfibrinogenemia can be associated with an increased risk of thrombosis, bleeding, or both. Here, we report a 36-year-old female with dysfibrinogenemia who experienced two successful pregnancies under thromboprophylaxis after cerebral venous sinus thrombosis (CVST)
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Veranstaltung des BDDH im Rahmen der 67. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung in Frankfurt Hämostaseologie (IF 3.2) Pub Date : 2023-06-20 Christoph Sucker, Jürgen Koscielny
Im Rahmen der 67. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung (GTH) in Frankfurt veranstaltete der BDDH am 23.2.2022 nach der Mitgliederversammlung eine öffentliche Sitzung zu berufspolitischen Themen. Die Sitzung war sehr gut besucht, wobei neben BDDH- Mitgliedern auch andere interessierte Ärzte und Vertreter der Industrie teilnahmen. In dieser Ausgabe der „BDDH News“ berichten
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Schwere nächtliche Hypoxie erhöht Wahrscheinlichkeit einer unprovozierten venösen Thromboembolie Hämostaseologie (IF 3.2) Pub Date : 2023-06-20
Die obstruktive Schlafapnoe (OSA) wurde zunächst als Ursache für Schäfrigkeit, neurologische Verhaltensstörungen und eine Beeinträchtigung der Lebensqualität angesehen. Darüber hinaus wirkt sich die OSA auch auf zahlreiche Erkrankungen aus, darunter Stoffwechsel- und Herz-Kreislauf-Erkrankungen sowie Krebs. Frühere Studien haben widersprüchliche Ergebnisse hinsichtlich des Zusammenhangs zwischen OSA
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Residuale Venenokklusion nach längerer Antikoagulanzien-Therapie nicht mit erneuten venösen Thromboembolien assoziiert Hämostaseologie (IF 3.2) Pub Date : 2023-06-20
Eine residuale Venenokklusion (RVO) gilt als Risikofaktor für ein erneutes Auftreten und möglicherweise auch für andere klinische Ereignisse nach einer tiefen Venenthrombose (TVT). Die aktuellen Leitlinien unterstützen eine auf die RVO abgestimmte Dauer der Antikoagulanzien-Therapie nicht. Aktuelle Daten zu solchen Behandlungsstrategien sind kaum vorhanden.
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Genetic Analysis of Hereditary Coagulation Factor V Deficiency in Two Chinese Families Caused by Compound Heterozygous Mutations Hämostaseologie (IF 3.2) Pub Date : 2023-06-15 Yuan Chen, Ke Zhang, Yanhui Jin, Manlin Zeng, Kaiqi Jia, Lihong Yang, Mingshan Wang
Objective This study aims to provide a preliminary discussion of the molecular basis of FV deficiency caused by compound heterozygous mutations in two Chinese families. Methods Relative coagulation index was measured by the one-stage clotting method and the FV:Ag was measured by ELISA. All exons and flanking regions of the F5 gene were amplified by PCR and directly sequenced. ClustalX-2.1-win was used
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Rectus Sheath Hematoma in a Patient with Dual-Antiplatelet Therapy Including Ticagrelor: A Case Report Hämostaseologie (IF 3.2) Pub Date : 2023-05-19 Sinan Genç, Ahmet Sefa Yeter, Ahmet Burak Oğuz, Ayça Koca, Onur Polat, Müge Günalp Eneyli
Rectus sheath hematoma (RSH) is an uncommon cause of abdominal pain associated with several risk factors including trauma, asthma, chronic obstructive pulmonary disease, pregnancy, and anticoagulation as it can be iatrogenic. Dual-antiplatelet therapy (DAPT), combined usage of a P2Y12 receptor inhibitor and aspirin, is a cornerstone treatment for patients with acute coronary syndromes. Ticagrelor is
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Comparison of Performances among Four Bleeding-Prediction Scores in Elderly Cancer Patients with Venous Thromboembolism Hämostaseologie (IF 3.2) Pub Date : 2023-05-03 Shaozhi Xi, Chaoyang Liu, Shuihua Yu, Jingxuan Qiu, Shuibo He, Zhong Yi
The performances of RIETE, VTE-BLEED, SWITCO65 + , and Hokusai-VTE scores for predicting major bleeding events in hospitalized elderly cancer patients with venous thromboembolism (VTE) have not been evaluated. This study validated the performances of these scoring systems in a cohort of elderly cancer patients with VTE. Between June 2015 and March 2021, a total of 408 cancer patients (aged ≥ 65 years)
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Factor XIII and Endothelial Dysfunction in Patients with Systemic Sclerosis Hämostaseologie (IF 3.2) Pub Date : 2023-05-01 Sonja Alesci, Matthias Wahle, Andrea Himsel, Wolfgang Miesbach
Systemic sclerosis (SSc, scleroderma) is a severe autoimmune connective tissue disease which affects the skin and internal organs. There has been evidence that coagulation factor XIII (FXIII) has a positive impact on clinical results in patients with SSc. In a single-center cohort study, we investigated the relationship between coagulation FXIII, endothelial dysfunction, and skin infection in SSc.
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P2Y12-Inhibitoren hemmen Thrombozyten-induzierte Monozytenaktivierung Hämostaseologie (IF 3.2) Pub Date : 2023-04-26
Thrombozyten haben neben ihrer Rolle im Rahmen von Hämostase und Thrombose auch eine Mediatorfunktion bei thrombo-inflammatorischen Prozessen inne. In einer aktuellen experimentellen Studie konnte gezeigt werden, dass P2Y12-Inhibioren die Thrombozyten-induzierten Monozytenaktivierung abschwächen und damit antiinflammatorisch wirken.
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Orale Antikoagulation mit VKA: Nicht-CYP2C9-inhibierende SSRI einsetzen! Hämostaseologie (IF 3.2) Pub Date : 2023-04-26
Selektive Serotonin-Wiederaufnahmehemmer (SSRI) erhöhen bei Patienten, die unter einer oralen Antikoagulation mit einem Vitamin-K-Antagonisten (VKA) stehen, das Risiko für Blutungskomplikationen. Eine aktuelle Studie bestätigte den Zusammenhang erneut und lieferte Details zu einzelnen Substanzgruppen der SSRI und VKA.