HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/a-2215-8936
Herbert Schöchl, Felix C F Schmitt, Marc Maegele
{"title":"Pathophysiology of Trauma-Induced Coagulopathy.","authors":"Herbert Schöchl, Felix C F Schmitt, Marc Maegele","doi":"10.1055/a-2215-8936","DOIUrl":"10.1055/a-2215-8936","url":null,"abstract":"<p><p>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 shock and substantial tissue injury, resulting in endothelial damage, glycocalyx shedding, upregulated fibrinolysis, fibrinogen depletion, altered thrombin generation, and platelet dysfunction. Exogenous factors such as hypothermia, acidosis, hypokalemia, and dilution due to crystalloid and colloid fluid administration can further exacerbate TIC. Established TIC upon emergency room admission is a prognostic indicator and is strongly associated with poor outcomes. It has been shown that patients with TIC are prone to higher bleeding tendencies, increased requirements for allogeneic blood transfusion, higher complication rates such as multi-organ failure, and an almost fourfold increase in mortality. Thus, early recognition and individualized treatment of TIC is a cornerstone of initial trauma care. However, patients who survive the initial insult switch from hypocoagulability to hypercoagulability, also termed \"late TIC,\" with a high risk of developing thromboembolic complications.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/s-0044-1779290
Cihan Ay, Christoph Male
{"title":"GTH 2024: Building Bridges in Coagulation.","authors":"Cihan Ay, Christoph Male","doi":"10.1055/s-0044-1779290","DOIUrl":"10.1055/s-0044-1779290","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991939","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-01-08DOI: 10.1055/a-2215-9909
Florian Moik, Jakob M Riedl, Cornelia Englisch, Cihan Ay
{"title":"Update on Thrombosis Risk in Patients with Cancer: Focus on Novel Anticancer Immunotherapies.","authors":"Florian Moik, Jakob M Riedl, Cornelia Englisch, Cihan Ay","doi":"10.1055/a-2215-9909","DOIUrl":"10.1055/a-2215-9909","url":null,"abstract":"<p><p>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 with cancer is needed. Medical anticancer therapies have undergone a remarkable development in recent years with the advent of targeted and immunotherapeutic treatment options, including immune checkpoint inhibitors (ICI), chimeric antigen receptor (CAR) T-cell therapies and bispecific T-cell engagers (BiTEs). These developments have important implications for the accompanied risk of thromboembolic events in patients with cancer. First, the increased use of these highly effective therapies renders a growing proportion of patients with cancer at risk of thromboembolic events for a prolonged risk period due to an increase in patient survival despite advanced cancer stages. Second, potential direct cardiovascular toxicity and prothrombotic effect of novel anticancer immunotherapies are a matter of ongoing debate, with emerging reports suggesting a relevant risk of VTE and ATE associated with ICI, and relevant dysregulations of hemostasis in the frequently observed cytokine-release syndrome associated with BiTEs and CAR T-cell therapy. The aim of the present narrative review is to summarize the implications of the emerging use of anticancer immunotherapy for thromboembolic events in patients with cancer, and to provide an overview of available data on the rates and risk factors for VTE and ATE associated with ICI, CAR T-cell therapy, and BiTEs.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139405365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/a-2223-9484
Bernhard Lämmle, Karen Vanhoorelbeke, Johanna A Kremer Hovinga, Paul Knöbl
{"title":"100 Years of Thrombotic Thrombocytopenic Purpura: A Story of Death and Life.","authors":"Bernhard Lämmle, Karen Vanhoorelbeke, Johanna A Kremer Hovinga, Paul Knöbl","doi":"10.1055/a-2223-9484","DOIUrl":"10.1055/a-2223-9484","url":null,"abstract":"<p><p>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, the first beneficial treatment for TTP, to the elucidation of the pathophysiology, the discovery of ADAMTS13, the development of assays and targeted therapies up to the modern treatment concepts, that improved the outcome of TTP from an incurable disease to a well understood and treatable disorder.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/a-2219-6410
Benedetta Izzi, José J Fuster
{"title":"Clonal Hematopoiesis and Cardiovascular Risk: Atherosclerosis, Thrombosis, and beyond.","authors":"Benedetta Izzi, José J Fuster","doi":"10.1055/a-2219-6410","DOIUrl":"10.1055/a-2219-6410","url":null,"abstract":"<p><p>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 inflammatory responses. The insights gained from these studies are paving the way for the development of new personalized preventive care strategies against cardiovascular disease. Furthermore, available evidence also suggests a potential relevance of these mutation in the context of thrombosis, an area requiring thorough investigation. In this review, we provide an overview of our current understanding of this emerging cardiovascular risk factor, focusing on its relationship to atherosclerosis and thrombosis.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/s-0044-1782595
{"title":"PAS: Die Rolle von Natriumcitrat bei längerer Kaltlagerung von Thrombozyten.","authors":"","doi":"10.1055/s-0044-1782595","DOIUrl":"10.1055/s-0044-1782595","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-01Epub Date: 2024-02-28DOI: 10.1055/a-2178-6491
Waltraud C Schrottmaier, Alice Assinger
{"title":"The Concept of Thromboinflammation.","authors":"Waltraud C Schrottmaier, Alice Assinger","doi":"10.1055/a-2178-6491","DOIUrl":"10.1055/a-2178-6491","url":null,"abstract":"<p><p>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 influence inflammatory responses through interactions with immune cells, endothelium, or complement system. These interactions can lead to a state of heightened inflammation resulting from thrombotic processes, termed as thromboinflammation. This review aims to comprehensively summarize the existing knowledge of thromboinflammation and addressing its significance as a challenging clinical issue.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139991943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Digenic Inheritance of PROC and SERPINC1 Mutations Contributes to Multiple Sites Venous Thrombosis.","authors":"Xiangui Li, Jiabao Zhu, Fanzhen Lv, Wenqi Ma, Weimin Zhou, Wenwen Zhang","doi":"10.1055/a-2212-1565","DOIUrl":"https://doi.org/10.1055/a-2212-1565","url":null,"abstract":"<p><p>Venous thromboembolism (VTE) represents a worldwide health challenge, impacting millions of people each year. The genesis of venous thrombosis is influenced in part by genetic components. Hereditary thrombosis is described as a genetically determined susceptibility to VTE. In the present study, a male patient was referred to our department presenting with multiple venous thrombosis events in different locations. Given a lack of identifiable risk factors, we aimed to investigate the possible genetic factor underlying venous thrombosis. Whole-exome sequencing was employed to examine genes linked to inherited thrombophilia in the proband. Putative variants were subsequently confirmed through Sanger sequencing within the family. The proband was identified as carrying two genetic mutations. One is the novel c.400G > C (p.E134Q) mutation affecting the final nucleotide of exon 5 in the PROC gene, potentially impacting splicing. The other is a previously reported heterozygous nonsense variant c.1016G > A (p.W339X) in the SERPINC1 gene. The proband inherited the former from her mother and the latter from her father. The presence of digenic inheritance in the patient reflects the complex phenotype of venous thrombosis and demonstrates the significance of an unbiased approach to detect pathogenic variants, especially in patients with a high risk of hereditary thrombosis.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139472962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2023-12-04DOI: 10.1055/a-2197-9738
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
{"title":"Emicizumab for the Treatment of Acquired Hemophilia A: Consensus Recommendations from the GTH-AHA Working Group.","authors":"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","doi":"10.1055/a-2197-9738","DOIUrl":"https://doi.org/10.1055/a-2197-9738","url":null,"abstract":"<p><strong>Background: </strong> 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 demonstrated that emicizumab prevents bleeds and allows to postpone immunosuppression, which may influence future treatment strategies.</p><p><strong>Aim: </strong> To provide clinical practice recommendations on the use of emicizumab in AHA.</p><p><strong>Methods: </strong> A Delphi procedure was conducted among 33 experts from 16 German and Austrian hemophilia care centers. Statements were scored on a scale of 1 to 9, and agreement was defined as a score of ≥7. Consensus was defined as ≥75% agreement among participants, and strong consensus as ≥95% agreement.</p><p><strong>Results: </strong> Strong consensus was reached that emicizumab is effective for bleed prophylaxis and should be considered from the time of diagnosis (100% consensus). A fast-loading regimen of 6 mg/kg on day 1 and 3 mg/kg on day 2 should be used if rapid bleeding prophylaxis is required (94%). Maintenance doses of 1.5 mg/kg once weekly should be given (91%). Immunosuppression should be offered to patients on emicizumab if they are eligible based on physical status (97%). Emicizumab should be discontinued when remission of AHA is achieved (97%).</p><p><strong>Conclusion: </strong> These GTH consensus recommendations provide guidance to physicians on the use of emicizumab in AHA and follow the results of clinical trials that have shown emicizumab is effective in preventing bleeding in AHA.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2023-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138483537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}