HamostaseologiePub Date : 2024-10-01Epub Date: 2024-10-23DOI: 10.1055/s-0044-1791987
{"title":"iTTP in der Schwangerschaft: Erfolgreiche Behandlung mit Caplacizumab.","authors":"","doi":"10.1055/s-0044-1791987","DOIUrl":"https://doi.org/10.1055/s-0044-1791987","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142513323","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 : 2024-10-01Epub Date: 2024-10-23DOI: 10.1055/s-0044-1791988
{"title":"Komplementdysregulation als Biomarker für Post Covid.","authors":"","doi":"10.1055/s-0044-1791988","DOIUrl":"https://doi.org/10.1055/s-0044-1791988","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142513324","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 : 2024-10-01Epub Date: 2024-07-01DOI: 10.1055/a-2330-9112
Dino Mehic, Alice Assinger, Johanna Gebhart
{"title":"Utility of Global Hemostatic Assays in Patients with Bleeding Disorders of Unknown Cause.","authors":"Dino Mehic, Alice Assinger, Johanna Gebhart","doi":"10.1055/a-2330-9112","DOIUrl":"10.1055/a-2330-9112","url":null,"abstract":"<p><p>Bleeding disorder of unknown cause (BDUC) is a diagnosis of exclusion after exhaustive evaluation of plasmatic coagulation and platelet function. This review explores the utility of global hemostatic assays as confirmatory tests and in elucidating the pathophysiology of BDUC. Unlike traditional hemostatic tests that focus on coagulation factors, global assays are conducted both in plasma and also whole blood. These assays provide a more comprehensive understanding of the cell-based model of coagulation, aid in the identification of plasmatic factor abnormalities that may reduce hemostatic capacity, and allow for the assessment of impaired platelet-endothelial interactions under shear stress, as well as hyperfibrinolytic states. While clinical tests such as skin bleeding time and global assays such as PFA-100 exhibit limited diagnostic capacity, the role of viscoelastic testing in identifying hemostatic dysfunction in patients with BDUC remains unclear. Thrombin generation assays have shown variable results in BDUC patients; some studies demonstrate differences compared with healthy controls or reference values, whereas others question its clinical utility. Fibrinolysis assessment in vitro remains challenging, with studies employing euglobulin clot lysis time, plasma clot lysis time, and fluorogenic plasmin generation yielding inconclusive or conflicting results. Notably, recent studies suggest that microfluidic analysis unveils shear-dependent platelet function defects in BDUC patients, undetected by conventional platelet function assays. Overall, global assays might be helpful for exploring underlying hemostatic impairments, when conventional hemostatic laboratory tests yield no results. However, due to limited data and/or discrepant results, further research is needed to evaluate the utility of global assays as screening tools.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141478019","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 : 2024-10-01Epub Date: 2024-10-23DOI: 10.1055/a-2374-2903
Johannes Thaler, Carla Tripisciano, Rienk Nieuwland
{"title":"Investigations on the Hemostatic Potential of Physiological Body Fluids.","authors":"Johannes Thaler, Carla Tripisciano, Rienk Nieuwland","doi":"10.1055/a-2374-2903","DOIUrl":"https://doi.org/10.1055/a-2374-2903","url":null,"abstract":"<p><p>Current blood coagulation models consider the interactions between blood, the vessel wall, and other tissues that expose tissue factor (TF), the main initiator of coagulation. A potential role of body fluids other than blood is generally not considered. In this review, we summarize the evidence that body fluids such as mother's milk saliva, urine, semen, and amniotic fluid trigger coagulation. The ability of these body fluids to trigger coagulation is explained by the presence of extracellular vesicles (EVs). These EVs expose extrinsic tenase complexes (i.e., complexes of TF and activated factor VII) that can trigger coagulation. Why these body fluids share this activity, however, is unknown. Possible explanations are that these body fluids contribute to hemostatic protection and/or to the regulation of the epithelial barrier function. Further investigations may help understand the underlying cellular and biochemical pathways regulating or contributing to coagulation and innate immunity, which may be directly relevant to medical conditions such as gastrointestinal bleeding and chronic inflammatory bowel disease.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142513322","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 : 2024-10-01Epub Date: 2024-10-23DOI: 10.1055/s-0044-1791986
{"title":"Die Gesellschaft für Thrombose- und Hämostaseforschung e.V. informiert.","authors":"","doi":"10.1055/s-0044-1791986","DOIUrl":"https://doi.org/10.1055/s-0044-1791986","url":null,"abstract":"","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142513321","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 : 2024-10-01Epub Date: 2023-10-31DOI: 10.1055/a-2159-8722
Mathias Haargaard Nielsen, Mustafa Vakur Bor
{"title":"Management of Recurrent Venous Thromboembolism in Severe Immune Thrombocytopenia: A Case Report and a Review of the Literature.","authors":"Mathias Haargaard Nielsen, Mustafa Vakur Bor","doi":"10.1055/a-2159-8722","DOIUrl":"10.1055/a-2159-8722","url":null,"abstract":"<p><p>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 × 10<sup>9</sup>/L. We further review studies reporting venous thromboembolism (VTE) in patients with severe ITP (≤ 35 × 10<sup>9</sup>/L) and identified 14 patients highlighting VTE risk factors and management of these patients. The present case had several risk factors for VTE (previous DVT, obesity, heterozygosity for factor V Leiden mutation, and previous splenectomy). The patient was initially treated with low-molecular-weight heparin followed by long-term apixaban treatment. The literature review together with our case demonstrates that VTE in severe ITP (≤ 35 × 10<sup>9</sup>/L) can occur in patients with VTE risk factors and antithrombotic management of these patients can be achieved without bleeding depending on severity of thrombocytopenia either by full or reduced dose of anticoagulation together with ITP therapy.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71429275","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 : 2024-09-03DOI: 10.1055/a-2347-6507
Cornelia Englisch, Nikola Vladic, Cihan Ay
{"title":"Bleeding Risk in Patients with Cancer.","authors":"Cornelia Englisch, Nikola Vladic, Cihan Ay","doi":"10.1055/a-2347-6507","DOIUrl":"https://doi.org/10.1055/a-2347-6507","url":null,"abstract":"<p><p>The hemostatic system and cancer display a tight interconnection, and hemostatic imbalance frequently occurs in patients with cancer. While extensive knowledge about thrombotic risk has been generated, less is known about bleeding risk and associated risk factors. However, bleeding risk is of high significance as patients with cancer frequently receive therapeutic anticoagulation for various indications and/or are candidates for primary thromboprophylaxis. The risk of bleeding in patients with cancer is variable and difficult to assess in clinical practice. Certain clinical settings such as hospitalization, specific underlying risk factors (e.g., tumor type), and medications (e.g., anticoagulation) can contribute to the individual bleeding risk of a patient with cancer. In addition, some dynamic factors such as platelet count or kidney function have an impact. Particularly, data on baseline risk of bleeding are lacking to allow for risk assessment in cancer patients without anticoagulation. In contrast, risk assessment models for the prediction of bleeding events in cancer patients receiving anticoagulation have been developed; however, these have yet to be validated. The recognition of the importance of bleeding risk in cancer patients is growing, leading to an increasing number of studies investigating and reporting bleeding complications. As study designs and reporting of bleeding events vary, it is challenging to offer a clear synthesis of evidence. In this narrative review, we provide an overview of currently available data about incidence, risk factors, and clinical impact of bleeding events in patients with cancer, and critically review risk assessment models for bleeding in cancer patients during anticoagulant therapy.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142127420","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 : 2024-08-01Epub Date: 2024-07-11DOI: 10.1055/a-2315-6206
Philip Kienzl, Julia Deinsberger, Benedikt Weber
{"title":"Chronic Venous Disease: Pathophysiological Aspects, Risk Factors, and Diagnosis.","authors":"Philip Kienzl, Julia Deinsberger, Benedikt Weber","doi":"10.1055/a-2315-6206","DOIUrl":"10.1055/a-2315-6206","url":null,"abstract":"<p><p>Chronic venous disease (CVD) is highly prevalent in the general population and encompasses a range of pathological and hemodynamic changes in the veins of the lower extremities. These alterations give rise to a variety of symptoms, with more severe forms resulting in venous ulceration, which causes morbidity and high socioeconomic burden. The origins and underlying mechanisms of CVD are intricate and multifaceted, involving environmental factors, genetics, hormonal factors, and immunological factors that bring about structural and functional alterations in the venous system. This review offers the latest insights into the epidemiology, pathophysiology, and risk factors of CVD, aiming to provide a comprehensive overview of the current state of knowledge. Furthermore, the diagnostic approach for CVD is highlighted and current diagnostic tools are described.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141592220","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 : 2024-08-01Epub Date: 2024-06-26DOI: 10.1055/a-2247-4209
Jennifer Gebetsberger, Werner Streif, Christof Dame
{"title":"Update on the Use of Thrombopoietin-Receptor Agonists in Pediatrics.","authors":"Jennifer Gebetsberger, Werner Streif, Christof Dame","doi":"10.1055/a-2247-4209","DOIUrl":"10.1055/a-2247-4209","url":null,"abstract":"<p><p>This review summarizes the rationale and current data on the use of thrombopoietin receptor agonists (TPO-RAs) for treating severe thrombocytopenia in infants, children, and adolescents. It focuses on substances that have been approved by the U.S. Food and Drug Administration (FDA) and European Medicines Agency (EMA) for pediatric patients. Romiplostim and eltrombopag are already established as second-line treatment for persistent or chronic immune thrombocytopenia (ITP). As in adults, TPO-RAs are currently also evaluated in severe aplastic anemia (SAA), chemotherapy-induced thrombocytopenia (CIT), myelodysplastic syndromes (MDS), and poor engraftment after hematopoietic stem cell transplantation in pediatric and adolescent patients. Moreover, studies on the implication of TPO-RA in treating rare inherited thrombocytopenias, such as Wiskott-Aldrich syndrome (WAS), congenital amegakaryocytic thrombocytopenia (CAMT), or <i>MYH9</i>-associated thrombocytopenia, deserve future attention. Current developments include testing of avatrombopag and lusutrombopag that are approved for the treatment of thrombocytopenia associated with chronic liver disease (CLD) in adult patients. In pediatric and adolescent medicine, we expect in the near future a broader use of TPO-RAs as first-line treatment in primary ITP, thereby considering immunomodulatory effects that increase the rate of sustained remission off-treatment, and a selective use in rare inherited thrombocytopenias based on current clinical trials.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":null,"pages":null},"PeriodicalIF":2.7,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141460879","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}