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Hämostaseologie
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ISSN (Print) 0720-9355 - ISSN (Online) 2567-5761
Published by Thieme Publishing Group

[233 journals]

GTH 2022: Sustainable Collaboration
- Hamostaseologie 2022; 42: 007-008
  DOI: 10.1055/a-1694-4829
  
  Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
  
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  Inhaltsverzeichnis Volltext
  Hamostaseologie 2022; 42: 007-0082022-02-23T00:00:00+0100
  Issue No: Vol. 42, No. 01 (2022)
Treatment of Atypical Clots
- Authors: Ageno; Walter
  Pages: 010 - 018
  Abstract: The term unusual site thrombosis refers to the occurrence of venous thromboembolism outside of the lower limbs and the pulmonary arteries. Unusual site thrombosis can virtually affect any venous segment, with the most common and/or clinically relevant sites including the upper limbs veins, the retinal veins, the splanchnic veins and the cerebral veins. Clinical features are obviously heterogeneous, while many predisposing factors are common, though with different prevalence among the various disorders. Antithrombotic therapy is prescribed to most patients, but the type, intensity, and duration of treatment vary both within and among patient groups. Increasing evidence is supporting the use of the direct oral anticoagulants in patients with upper limb deep vein thrombosis, cerebral vein thrombosis, and with splanchnic vein thrombosis. This article will review available evidence on the treatment of venous thrombosis occurring in unusual sites.
  Citation: Hamostaseologie 2022; 42: 010-018
  PubDate: 2022-02-23T00:00:00+0100
  DOI: 10.1055/a-1669-3336
  Issue No: Vol. 42, No. 01 (2022)
Diagnosis of Platelet Function Disorders: A Challenge for Laboratories
- Authors: Wagner; Miriam, Uzun, Günalp, Bakchoul, Tamam, Althaus, Karina
  Pages: 036 - 045
  Abstract: In patients with normal plasmatic coagulation and bleeding tendency, platelet function defect can be assumed. Congenital platelet function defects are rare. Much more commonly they are acquired. The clinical bleeding tendency of platelet function defects is heterogeneous, which makes diagnostic approaches difficult. During the years, a large variety of tests for morphological phenotyping and functional analysis have been developed. The diagnosis of platelet function defects is based on standardized bleeding assessment tools followed by a profound morphological evaluation of the platelets. Platelet function assays like light transmission aggregation, luminoaggregometry, and impedance aggregometry followed by flow cytometry are commonly used to establish the diagnosis in these patients. Nevertheless, despite great efforts, standardization of these tests is poor and in most cases, quality control is lacking. In addition, these tests are still limited to specialized laboratories. This review summarizes the approaches to morphologic phenotyping and platelet testing in patients with suspected platelet dysfunction, beginning with a standardized bleeding score and ending with flow cytometry testing. The diagnosis of a functional defect requires a good collaboration between the laboratory and the clinician.
  Citation: Hamostaseologie 2022; 42: 036-045
  PubDate: 2022-02-23T00:00:00+0100
  DOI: 10.1055/a-1700-7036
  Issue No: Vol. 42, No. 01 (2022)
Thrombophilia, Thrombosis and Thromboprophylaxis in Pregnancy: For What
and in Whom'
- Authors: Middeldorp; Saskia, Naue, Christiane, Köhler, Christina
  Pages: 054 - 064
  Abstract: Compared with nonpregnant women, pregnancy carries a four- to fivefold higher risk of venous thromboembolism (VTE). Despite increasing use of heparin prophylaxis in identified high-risk patients, pulmonary embolism still is the leading cause of maternal mortality in the western world. However, evidence on optimal use of thromboprophylaxis is scarce. Thrombophilia, the hereditary or acquired tendency to develop VTE, is also thought to be associated with complications in pregnancy, such as recurrent miscarriage and preeclampsia. In this review, the current evidence on optimal thromboprophylaxis in pregnancy is discussed, focusing primarily on VTE prevention strategies but also discussing the potential to prevent recurrent pregnancy complications with heparin in pregnant women with thrombophilia.
  Citation: Hamostaseologie 2022; 42: 054-064
  PubDate: 2022-02-23T00:00:00+0100
  DOI: 10.1055/a-1717-7663
  Issue No: Vol. 42, No. 01 (2022)
An Update on Safe Anticoagulation
- Authors: Mailer; Reiner K., Kuta, Piotr, Renné, Thomas
  Pages: 065 - 072
  Abstract: Blood coagulation is essential to maintain the integrity of a closed circulatory system (hemostasis), but also contributes to thromboembolic occlusion of vessels (thrombosis). Thrombosis may cause deep vein thrombosis, pulmonary embolism, myocardial infarction, peripheral artery disease, and ischemic stroke, collectively the most common causes of death and disability in the developed world. Treatment for the prevention of thromboembolic diseases using anticoagulants such as heparin, coumarins, thrombin inhibitors, or antiplatelet drugs increase the risk of bleeding and are associated with an increase in potentially life-threatening hemorrhage, partially offsetting the benefits of reduced coagulation. Thus, drug development aiming at novel targets is needed to provide efficient and safe anticoagulation. Within the last decade, experimental and preclinical data have shown that some coagulation mechanisms principally differ in thrombosis and hemostasis. The plasma contact system protein factors XII and XI, high-molecular-weight kininogen, and plasma kallikrein specifically contribute to thrombosis, however, have minor, if any, role in hemostatic coagulation mechanisms. Inherited deficiency in contact system proteins is not associated with increased bleeding in humans and animal models. Therefore, targeting contact system proteins provides the exciting opportunity to interfere specifically with thromboembolic diseases without increasing the bleeding risk. Recent studies that investigated pharmacologic inhibition of contact system proteins have shown that this approach provides efficient and safe thrombo-protection that in contrast to classical anticoagulants is not associated with increased bleeding risk. This review summarizes therapeutic and conceptual developments for selective interference with pathological thrombus formation, while sparing physiologic hemostasis, that enables safe anticoagulation treatment.
  Citation: Hamostaseologie 2022; 42: 065-072
  PubDate: 2022-02-23T00:00:00+0100
  DOI: 10.1055/a-1717-7958
  Issue No: Vol. 42, No. 01 (2022)
When and How to Combine Antiplatelet and Anticoagulant Drugs'
- Authors: Espinola-Klein; Christine
  Pages: 073 - 079
  Abstract: Antiplatelet and anticoagulant drugs work at different places in the coagulation system. Antiplatelet drugs are usually indicated in patients with atherosclerosis. Anticoagulant drugs are mostly used in patients with atrial fibrillation, venous thromboembolism, or technical heart valves. In some clinical situations, combination of antiplatelet and anticoagulant therapy can be indicated. The most recent situations are a more intensive antithrombotic therapy for risk reduction in patients with atherosclerosis and temporary addition of antiplatelet drugs in patients with indication for long-term anticoagulation. Temporary combination of antiplatelet and anticoagulant drugs is usually necessary after coronary intervention in patients with atrial fibrillation. In patients with high-risk atherosclerosis, the combination of low-dose rivaroxaban and aspirin reduces major adverse cardiovascular events (myocardial infarction, stroke, cardiovascular death) and major adverse limb events. But every combination of antiplatelet and antithrombotic drugs can increase bleeding risk. Therefore, a careful assessment of thrombotic versus bleeding risk is necessary for each patient.
  Citation: Hamostaseologie 2022; 42: 073-079
  PubDate: 2022-02-23T00:00:00+0100
  DOI: 10.1055/a-1724-4922
  Issue No: Vol. 42, No. 01 (2022)
Neuigkeiten zur Ablösung der geplanten Ablösung der
In-Vitro-Diagnostika-Richtlinie durch die In-Vitro-Diagnostika-Verordnung
(IVDR) 05/2022 und Plasmaversorgung in Deutschland/EU und Fortführung der
konzertierten Aktion des BDDH mit den Selbsthilfegruppen
- Hamostaseologie 2022; 42: 081-083
  DOI: 10.1055/a-1694-4640
  
  Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
  
  Artikel in Thieme eJournals:
  Inhaltsverzeichnis Volltext
  Hamostaseologie 2022; 42: 081-0832022-02-23T00:00:00+0100
  Issue No: Vol. 42, No. 01 (2022)
Assessment and Monitoring of Coagulation in Patients with COVID-19: A
Review of Current Literature
- Authors: Uzun; Günalp, Althaus, Karina, Hammer, Stefanie, Bakchoul, Tamam
  Abstract: Coagulation abnormalities are common in patients with COVID-19 and associated with high morbidity and mortality. It became a daily challenge to navigate through these abnormal laboratory findings and deliver the best possible treatment to the patients. The unique character of COVID-19-induced coagulopathy necessitates not only a dynamic follow-up of the patients in terms of hemostatic findings but also the introduction of new diagnostic methods to determine the overall function of the coagulation system in real time. After the recognition of the high risk of thromboembolism in COVID-19, several professional societies published their recommendations regarding anticoagulation in patients with COVID-19. This review summarizes common hemostatic findings in COVID-19 patients and presents the societal recommendations regarding the use of coagulation laboratory findings in clinical decision-making. Although several studies have investigated coagulation parameters in patients with COVID-19, the methodological shortcomings of published studies as well as the differences in employed anticoagulation regimens that have changed over time, depending on national and international guidelines, limit the applicability of these findings in other clinical settings. Accordingly, evidence-based recommendations for diagnostics during acute COVID-19 infection are still lacking. Future studies should verify the role of coagulation parameters as well as viscoelastic methods in the management of patients with COVID-19.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-04-27T00:00:00+01:00
  DOI: 10.1055/a-1755-8676
Interventional Treatment Options in Children with Extracranial Vascular
Malformations
- Authors: Schmidt; Vanessa F., Olivieri, Martin, Häberle, Beate, Masthoff, Max, Deniz, Sinan, Sporns, Peter B., Wohlgemuth, Walter A., Wildgruber, Moritz
  Abstract: Extracranial vascular malformations vary greatly and belong to the complex field of orphan diseases and can involve all segments of the vascular tree: arteries, capillaries, and veins, and similarly the lymphatic system. The classification according to the International Society for the Study of Vascular Anomalies (ISSVA) represents an important guidance for selecting appropriate therapy. Although many of the principles of endovascular treatment, including image-guided sclerotherapy and embolization, are similar in adult and pediatric practice, there are some distinct differences regarding the treatment of vascular malformations of children. Thus, it is crucial to involve longer-term plan about managing these chronic diseases and their impact on a growing child. This review provides a detailed overview over the clinical presentation of venous, lymphatic, and arteriovenous malformations in children and emphasizes the specifics of their interventional treatment options, including distinct pediatric dose limitations and procedure-related side effects.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-03-09T00:00:00+0100
  DOI: 10.1055/a-1728-5686
Cardiac and Cerebral Arterial Complications of Lemierre Syndrome: Results
from a Systematic Review and Individual Patient Data Meta-analysis
- Authors: Pleming; William, Barco, Stefano, Voci, Davide, Sacco, Clara, Zane, Federica, Granziera, Serena, Corsi, Gabriele, Konstantinides, Stavros V., Kucher, Nils, Pecci, Alessandro, Valerio, Luca
  Abstract: Background Lemierre syndrome is a potentially life-threatening disease, which affects otherwise healthy young adults and adolescents. It is characterized by acute neck vein thrombosis and septic embolism, usually complicating a bacterial infection. Data on the syndrome are sparse, particularly concerning arterial complications. Methods We evaluated the frequency and patterns of cerebral arterial and cardiac involvement (“arterial complications”) in an individual patient level cohort of 712 patients, representing all cases described over the past 20 years in the medical literature who fulfilled the criteria: (1) bacterial infection in the neck/head site and (2) objectively confirmed thrombotic complication or septic embolism. The study outcomes were defined as all-cause in-hospital deaths and the occurrence of clinical sequelae at discharge or in the postdischarge period. Results A total of 55 (7.7%) patients had an arterial complication. The most frequent arterial complications were carotid involvement (52.7%), stroke (38.2%), and pericardial complications (20%). Patients with an arterial involvement were more likely to be treated with a greater number of antibiotics (23 vs. 10%) and to receive anticoagulation. In addition, patients with arterial complications had a greater risk of all-cause death (n = 20/600, 3.3% vs. n = 6/52, 12%; odds ratio [OR]: 3.8; 95% confidence interval [CI]: 1.5–9.9) and late clinical sequelae (n = 49/580, 9.0% vs. n = 15/46, 35%; OR: 5.2; 95% CI: 2.65–10.37). Conclusions While Lemierre syndrome is known to be primarily characterized by venous thromboembolic events, our results suggest that local or distant arterial complications may occur in approximately one-tenth of patients and may be associated with a greater risk of long-term sequelae and death.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-03-07T00:00:00+0100
  DOI: 10.1055/a-1694-8723
High Prevalence of Plasminogen Activator Inhibitor-1 4G/5G Polymorphism
among Patients with Venous Thromboembolism in Kerala, India
- Authors: Tony; Ann Maria, Vinod, Vivek, Nambiar, Vivek, Krishnan, Sajitha, Biswas, Lalitha
  Abstract: Venous thromboembolism (VTE) is a multifactorial clotting disorder in which inherited and environmental factors synergistically contribute to its pathogenesis. The aim of this case–control study was to analyze the prevalence of hereditary thrombophilic risk factors, provoking and non-provoking environmental risk factors in patients with VTE from Kerala, India. We have observed a low prevalence of factor V Leiden (7%), prothrombin G20210A (2%), and prothrombin G20030A (2%) mutations and a high prevalence of plasminogen activator inhibitor-1 (PAI-1) 4G/5G (52%), PAI-1 4G/4G (24%) genotypes in the VTE patients (n = 147). Deficiency of anticoagulants, antithrombin (3.4%), and protein C (4.1%) was relatively low. None of the risk factors were observed in 17% of the patients. Majority of VTE patients were younger than 50 years with a median age of 43 years. In conclusion, our results indicate a high prevalence of PAI-1 4G/5G polymorphism among the VTE patients which is in concordance with previous studies in the Asian population. The PAI-1 4G/5G polymorphism could be a potential biomarker for assessing VTE risk, particularly among the Indian population.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-03-07T00:00:00+0100
  DOI: 10.1055/a-1733-2143
Experiences in Routine Genetic Analysis of Hereditary Hemorrhagic,
Thrombotic, and Platelet Disorders
- Authors: Pezeshkpoor; B., Oldenburg, J., Pavlova, A.
  Abstract: Hemostasis is a complex and tightly regulated system that attempts to maintain a homeostatic balance to permit normal blood flow, without bleeding or thrombosis. Hemostasis reflects the subtle balance between procoagulant and anticoagulant factors in the pathways of primary hemostasis, secondary hemostasis, and fibrinolysis. The major components in this interplay include the vascular endothelium, platelets, coagulation factors, and fibrinolytic factors. After vessel wall injury, the subendothelium is exposed to the blood stream, followed by rapid activation of platelets via collagen binding and von Willebrand factor–mediated platelet adhesion to the damaged vessel wall through platelet glycoprotein receptor Ib/IX/V. Activated platelets change their shape, release bioactive molecules from their granules, and expose negatively charged phospholipids on their surface. For a proper function of this process, an adequate number of functional platelets are required. Subsequently, a rapid generation of sufficient amounts of thrombin begins; followed by activation of the coagulation system and its coagulation factors (secondary hemostasis), generating fibrin that consolidates the platelet plug. To maintain equilibrium between coagulation and anticoagulation, the naturally occurring anticoagulants such as protein C, protein S, and antithrombin keep this process in balance. Deficiencies (inherited or acquired) at any level of this fine-tuned system result in pathologic bleedings or increased hypercoagulability states leading to thrombosis. This review will focus on genetic diagnosis of inherited bleeding, thrombotic, and platelet disorders, discussing strengths and limitations of existing diagnostic settings and genetic tools and highlight some important considerations necessary for clinical application.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-02-28T00:00:00+0100
  DOI: 10.1055/a-1726-4793
Novel Likely Pathogenic Variant in the A3 Domain of von Willebrand Factor
Leading to a Collagen-Binding Defect
- Authors: Fels; Salome, Boeckelmann, Doris, Glonnegger, Hannah, Büchsel, Martin, Zieger, Barbara
  Abstract: Von Willebrand disease (VWD) is the most prevalent congenital bleeding disorder. Diagnosis and classification of VWD is complex due to its heterogeneity regarding clinical manifestations and molecular genetic analysis. Genetic investigations became an inherent part of diagnosis and help distinguish different types/subtypes of VWD. Although many variants have been listed being causative for VWD, the genetic etiology remains undefined in a lot of patients. We report about two siblings with severely reduced values for von Willebrand factor collagen-binding activity (VWF:CB). Genetic analysis using panel sequencing identified a heterozygous non-synonymous single nucleotide variant in exon 30. At the protein level, the alteration (p.Ser1731Leu) is located in the A3 collagen-binding domain. The amino acid position is already known to be important for collagen binding because p.Ser1731Thr has been reported to affect the VWF:CB.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-02-01T00:00:00+0100
  DOI: 10.1055/a-1701-2181
An Update on Laboratory Diagnostics in Haemophilia A and B
- Authors: Müller; Jens, Miesbach, Wolfgang, Prüller, Florian, Siegemund, Thomas, Scholz, Ute, Sachs, Ulrich J.
  Abstract: Haemophilia A (HA) and B (HB) are X-linked hereditary bleeding disorders caused by lack of activity of coagulation factors VIII (FVIII) or IX (FIX), respectively. Besides conventional products, modern replacement therapies include FVIII or FIX concentrates with an extended half-life (EHL-FVIII/FIX). Two main strategies for measuring plasma FVIII or FIX activity are applied: the one-stage clotting assay (OSCA) and the chromogenic substrate assay (CSA), both calibrated against plasma (FVIII/FIX) standards. Due to the structural modifications of EHL-FVIII/FIX, reagent-dependent assay discrepancies have been described when measuring the activity of these molecules. Assay discrepancies have also been observed in FVIII/FIX gene therapy approaches. On the other hand, nonfactor replacement by the bispecific antibody emicizumab, a FVIIIa-mimicking molecule, artificially shortens activated partial thromboplastin time–based clotting times, making standard OSCAs inapplicable for analysis of samples from patients treated with this drug. In this review, we aim to give an overview on both, the currently applied and future therapies in HA and HB with or without inhibitors and corresponding test systems suitable for accompanying diagnostics.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-02-01T00:00:00+0100
  DOI: 10.1055/a-1665-6232
Successful Secondary Endovascular Intervention in Pediatric Patients with
Venous Thromboembolic Events
- Authors: Glonnegger; Hannah, Zieger, Barbara, Grohmann, Jochen, Freund, Gabriele, Zeller, Thomas, Uhl, Markus, Stiller, Brigitte
  Abstract: Background In the past, pediatric patients with venous thromboembolic events (VTE) were treated with low-molecular-weight heparin (LMWH) which was successful in around 70% of the cases. However, anticoagulation alone might not restore patency in all patients, and advanced therapeutic options to prevent postthrombotic syndrome are needed. During recent years, endovascular interventions have become a treatment option for pediatric patients with persistent thrombotic occlusion, not only in life- or limb-threatening VTE. Methods We evaluated 12 consecutive patients (11–17 years) with newly diagnosed VTE being treated at our department during the last 4 years (2017–2020). In case follow-up examination showed persistent venoocclusion under anticoagulation, patients received secondary interventional therapy like recanalization, percutaneous transluminal angioplasty with or without catheter-directed thrombolysis, and stenting. Patients with no clinical signs of venoocclusion or regredient thrombosis in imaging examination received anticoagulation alone. Results Six of 12 (50%) patients underwent catheter intervention. Median time from diagnosis to intervention was 4 months (0–12 months). Reintervention was necessary in one (8%) case and complete recanalization failed in one (8%) case. There were no major bleeding events or other major postinterventional complications, no acute or late local recurrence, and all patients reported clinical improvement after the procedure. Conclusion If endovascular intervention is used in teenage patients with persistent symptomatic VTE, reduction of postthrombotic symptoms is possible, even if intervention is performed secondary to failure of anticoagulation. Multidisciplinary treatment decisions can be based on the clinical course and follow-up imaging.
  Citation: Hamostaseologie ; : -
  PubDate: 2022-01-13T00:00:00+0100
  DOI: 10.1055/a-1701-2098
Laudatio auf Rüdiger E. Scharf
- Pages: 425 - 426
  Abstract: Verehrte Leserinnen und Leser,die Ihnen vorliegende Ausgabe der Hämostaseologie – Progress in Haemostasis dient neben der gewohnt hochka-rätigen Informationsübermittlung als Festschrift anlässlich des 70. Geburtstags von Universitätsprofessor Dr. med. Rüdiger Eberhard Scharf, des langjährigen Herausgebers. Er ist emeritierter Lehrstuhlinhaber und ehemaliger Direktor des Instituts für Hämostaseologie, Hämotherapie und Transfusionsmedizin des Universitätsklinikums der Heinrich-Heine-Universität in Düsseldorf.
  Citation: Hamostaseologie 2021; 41: 425-426
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1370-0394
  Issue No: Vol. 41, No. 06 (2021)
Thromboinflammation as a Driver of Venous Thromboembolism
- Authors: Gauchel; Nadine, Krauel, Krystin, Hamad, Muataz Ali, Bode, Christoph, Duerschmied, Daniel
  Pages: 428 - 432
  Abstract: Thrombus formation has been identified as an integral part in innate immunity, termed immunothrombosis. Activation of host defense systems is known to result in a procoagulant environment. In this system, cellular players as well as soluble mediators interact with each other and their dysregulation can lead to the pathological process of thromboinflammation. These mechanisms have been under intensified investigation during the COVID-19 pandemic. In this review, we focus on the underlying mechanisms leading to thromboinflammation as one trigger of venous thromboembolism.
  Citation: Hamostaseologie 2021; 41: 428-432
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1661-0257
  Issue No: Vol. 41, No. 06 (2021)
Anti-inflammatory Strategies in Atherosclerosis
- Authors: Bugger; Heiko, Zirlik, Andreas
  Pages: 433 - 442
  Abstract: Atherosclerotic vascular disease and its related complications are the major cause of mortality in Western societies. Atherosclerosis is a chronic inflammatory disease of the arterial wall triggered by traditional and nontraditional risk factors and mediated by inflammatory and immune responses. Recent clinical trials provided compelling evidence corroborating that atherosclerosis is an inflammatory disease and demonstrated efficacy of anti-inflammatory interventions in reducing cardiovascular events and mortality. Traditional risk factors drive vascular inflammation, further justifying the instrumental role of intensified risk factor management in attenuating and preventing atherosclerotic disease and complications. Promising therapeutic approaches specifically related to inhibition of inflammation span traditional anti-inflammatory drugs, specific immunomodulation, and development of vaccination against atherosclerotic disease. Here, we review the inflammatory component in atherogenesis, the available evidence from clinical trials evaluating efficacy of therapeutic anti-inflammatory interventions in patients with high cardiovascular risk, and discuss potential future targets for anti-inflammatory or immune modulatory treatment in atherosclerotic cardiovascular disease.
  Citation: Hamostaseologie 2021; 41: 433-442
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1661-0020
  Issue No: Vol. 41, No. 06 (2021)
A Vicious Circle of Clonal Haematopoiesis of Indeterminate Potential and
Cardiovascular Disease
- Authors: Ehlert; Carolin A., Hilgendorf, Ingo
  Pages: 443 - 446
  Abstract: Clonal haematopoiesis of indeterminate potential (CHIP) represents a recently identified overlap between cancer and cardiovascular disease (CVD). CHIP develops as a result of certain acquired somatic mutations that predispose to leukaemia, but clinically even more prevalent, associate with increased risk for CVD and CVD-related death. Experimental studies suggest a causal role for CHIP aggravating inflammatory processes in CVD, and recent epidemiologic and genetic studies indicate that classical CVD risk factors may increase the risk of acquiring CHIP driver mutations, thus fuelling a vicious circle. The potential mechanism underlying the associative link between CHIP and CVD and mortality has been the focus of a few recent excellent experimental and observational studies which are summarized and discussed in this concise non-systematic review article. These data support a pathomechanistic view of a spiralling vicious circle in which CHIP aggravates the inflammatory immune response in CVD, and CVD-driven elevated haematopoietic activity promotes CHIP development.
  Citation: Hamostaseologie 2021; 41: 443-446
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1576-4059
  Issue No: Vol. 41, No. 06 (2021)
Modulating Autoimmunity against LDL: Development of a Vaccine against
Atherosclerosis
- Authors: Marchini; Timoteo, Abogunloko, Tijani, Wolf, Dennis
  Pages: 447 - 457
  Abstract: Atherosclerosis is a chronic inflammatory disease of the arterial wall that leads to the build-up of occluding atherosclerotic plaques. Its clinical sequelae, myocardial infarction and stroke, represent the most frequent causes of death worldwide. Atherosclerosis is a multifactorial pathology that involves traditional risk factors and chronic low-grade inflammation in the atherosclerotic plaque and systemically. This process is accompanied by a strong autoimmune response that involves autoreactive T cells in lymph nodes and atherosclerotic plaques, as well as autoantibodies that recognize low-density lipoprotein (LDL) and its main protein component apolipoprotein B (ApoB). In the past 60 years, numerous preclinical observations have suggested that immunomodulatory vaccination with LDL, ApoB, or its peptides has the potential to specifically dampen autoimmunity, enhance tolerance to atherosclerosis-specific antigens, and protect from experimental atherosclerosis in mouse models. Here, we summarize and discuss mechanisms, challenges, and therapeutic opportunities of immunomodulatory vaccination and other strategies to enhance protective immunity in atherosclerosis.
  Citation: Hamostaseologie 2021; 41: 447-457
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1661-1908
  Issue No: Vol. 41, No. 06 (2021)
Editorial
- Pages: 459 - 459
  Abstract: Dear colleagues,With this issue of HÄMOSTASEOLOGIE – Progress in Haemostasis it is time to deeply thank Prof Rüdiger Scharf for his accomplishments and his engagement as the journal's Editor-in-Chief (EIC) for more than 10 years.
  Citation: Hamostaseologie 2021; 41: 459-459
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1694-5259
  Issue No: Vol. 41, No. 06 (2021)
Pathogenic Aspects of Inherited Platelet Disorders
- Authors: Boeckelmann; Doris, Glonnegger, Hannah, Sandrock-Lang, Kirstin, Zieger, Barbara
  Pages: 460 - 468
  Abstract: Inherited platelet disorders (IPDs) constitute a large heterogeneous group of rare bleeding disorders. These are classified into: (1) quantitative defects, (2) qualitative disorders, or (3) altered platelet production rate disorders or increased platelet turnover. Classically, IPD diagnostic is based on clinical phenotype characterization, comprehensive laboratory analyses (platelet function analysis), and, in former times, candidate gene sequencing. Today, molecular genetic analysis is performed using next-generation sequencing, mostly by targeting enrichment of a gene panel or by whole-exome sequencing. Still, the biochemical and molecular genetic characterization of patients with congenital thrombocytopathias/thrombocytopenia is essential, since postoperative or posttraumatic bleeding often occurs due to undiagnosed platelet defects. Depending upon the kind of surgery or trauma, this bleeding may be life-threatening, e.g., after tonsillectomy or in brain surgery. Undiagnosed platelet defects may lead to additional surgery, hysterectomy, pulmonary bleeding, and even resuscitation. In addition, these increased bleeding symptoms can lead to wound healing problems. Only specialized laboratories can perform the special platelet function analyses (aggregometry, flow cytometry, or immunofluorescent microscopy of the platelets); therefore, many IPDs are still undetected.
  Citation: Hamostaseologie 2021; 41: 460-468
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1665-6249
  Issue No: Vol. 41, No. 06 (2021)
Type 2B von Willebrand Disease: Early Manifestation as Neonatal
Thrombocytopenia
- Authors: Kranzhöfer; David, Pavlova, Anna, Schneider, Hendryk, Franck, Peter, Glonnegger, Hannah, Büchsel, Martin, Yoshimi-Nöllke, Ayami, Oldenburg, Johannes, Zieger, Barbara
  Pages: 469 - 474
  Abstract: Here, we report about a preterm female newborn with a prolonged course of severe thrombocytopenia and hematomas. The family history was positive for von Willebrand disease type 2B (VWD 2B). Diagnosis of VWD 2B was identified analyzing von Willebrand factor (VWF) parameters (VWF:antigen, VWF:activity, VWF multimer analyses) and performing light transmission aggregometry (with half concentration of ristocetin). In addition, the diagnosis was confirmed by molecular genetic analysis: identification of a disease-causing missense mutation (Val1316Met) in the VWF gene associated with a severe course of VWD 2B, which had been previously reported. Treatment with a VWF-containing plasma concentrate was initiated. Because the combination of prematurity and very low platelet count is often associated with intracranial bleeding, at the beginning platelet concentrates were transfused. Fortunately, the patient did not develop serious bleeding episodes. Interestingly, the patient had a mutation in the VWF gene, which had been described to be associated with aggravation of thrombocytopenia especially in stressful situations. Therefore, we replaced venous blood withdrawals by capillary blood samplings when possible and, consequently, we observed an increase of the platelet count after this change in management. At the age of 2 months, the patient was discharged after stabilization of the platelet count without any bleeding signs and without a need of long-term medication.
  Citation: Hamostaseologie 2021; 41: 469-474
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1665-6185
  Issue No: Vol. 41, No. 06 (2021)
GTH News
- Hamostaseologie 2021; 41: 502-505
  DOI: 10.1055/s-0041-1741324
  
  Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany
  
  Artikel in Thieme eJournals:
  Inhaltsverzeichnis
  Hamostaseologie 2021; 41: 502-5052021-12-23T00:00:00+0100
  Issue No: Vol. 41, No. 06 (2021)
Ablösung der In-Vitro-Diagnostika-Richtlinie auf die
In-Vitro-Diagnostika-Verordnung (IVDR) 05/2022 und Programm für die
BDDH-Veranstaltung im Rahmen des GTH-Kongresses 2022 in Leipzig
- Authors: Sucker; Christoph, Kappert, Günther, Koscielny, Jürgen
  Pages: 506 - 508
  Abstract: Ablösung der In-Vitro-Diagnostika-Richtlinie auf die In-Vitro-Diagnostika-Verordnung (IVDR) 05/2022 (Christoph Sucker, Günther Kappert) Planmäßig soll am 26.05.2022 die bisher geltende In-Vitro-Diagnostika-Richtlinie durch die In-Vitro-Diagnostika-Verordnung (IVDR) ersetzt werden und würde dann an diesem Tag unmittelbar rechtlich wirksam.
  Citation: Hamostaseologie 2021; 41: 506-508
  PubDate: 2021-12-23T00:00:00+0100
  DOI: 10.1055/a-1370-0368
  Issue No: Vol. 41, No. 06 (2021)
Pulmonary Hypertension and COVID-19
- Authors: Castiglione; Laura, Droppa, Michal
  Abstract: Coronavirus disease 2019 (COVID-19) is a primary respiratory infectious disease, which can result in pulmonary and cardiovascular complications. From its first appearance in the city of Wuhan (China), the infection spread worldwide, leading to its declaration as a pandemic on March 11, 2020. Clinical research on SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) suggests that the virus may determine changes in the pulmonary hemodynamics through mechanisms of endothelial dysfunction, vascular leak, thrombotic microangiopathy, and venous thromboembolism that are similar to those leading to pulmonary hypertension (PH). Current available studies report echocardiographic signs of PH in approximately 12 to 13% of hospitalized patients with COVID-19. Those with chronic pulmonary obstructive disease, congestive heart failure, pulmonary embolism, and prior PH are at increased risk to develop or worsen PH. Evidence of PH seems to be associated with increased disease severity and poor outcome. Because of the importance of the pulmonary hemodynamics in the pathophysiology of COVID-19, there is growing interest in exploring the potential therapeutical benefits of inhaled vasodilators in patients with COVID-19. Treatment with inhaled nitric oxide and prostacyclin has shown encouraging results through improvement of systemic oxygenation, reduction of systolic pulmonary arterial pressure, and prevention of right ventricular failure; however, data from randomized control trials are still required.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-12-21T00:00:00+0100
  DOI: 10.1055/a-1661-0240
Anticoagulation Therapy in Cancer Patients with Thrombosis in the
Outpatient Sector of Germany (The CERTIFICAT Initiative)—German Practice
of Anticoagulation Therapy of Cancer Patients with Thrombosis
- Authors: Riess; Hanno, Kretzschmar, Albrecht, Heinken, Andreas, Mohebbi, Damon, May, Melanie, Schellong, Sebastian
  Abstract: Objective This article aims to investigate the reality of anticoagulation treatment for cancer patients with thrombosis in the outpatient sector of Germany. Methods For the analysis period 2012 to 2015, anonymized data from 4.1 million statutory insured patients were analyzed. Cancer patients with incident thrombosis and an outpatient prescription of anticoagulant drugs were identified and evaluated for three subsequent quarters with regard to anticoagulant use. Results A total of 7,313 cancer patients with incident thrombosis (ICD-10: I80*) were evaluated. About, 90% of patients with thromboses were diagnosed and treated in the ambulatory sector. More than 80% of the prescriptions were issued by general practitioners. And 57% of patients were anticoagulated predominantly (>50% of the time) with different low-molecular-weight heparins (LMWHs), 24% predominantly with vitamin K antagonists (VKAs), and 17% with direct oral anticoagulants (DOACs). Anticoagulants were prescribed for an average of 4.5 months. LMWH had a substantially longer prescription period (90–135 days) than VKA (53 days) or DOAC (47 days). Gastrointestinal bleeding in conjunction with hospitalization was documented in 1.76% of patients with a range of 1.3 to 3% for the different LMWHs. Conclusion The prescription practice documented by this representative and comprehensive evaluation demonstrates an anticoagulation duration in accordance with the guidelines, although the choice of the respective anticoagulant was often not in compliance with the contemporary label or guidelines.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-12-08T00:00:00+0100
  DOI: 10.1055/a-1554-4664
Platelet Dysregulation in the Pathobiology of COVID-19
- Authors: Mellema; Rebecca A., Crandell, Jacob, Petrey, Aaron C.
  Abstract: Coronavirus disease 2019 (COVID-19) encompasses a broad spectrum of clinical manifestations caused by infection with severe acute respiratory syndrome coronavirus 2.Patients with severe disease present with hyperinflammation which can affect multiple organs which often include observations of microvascular and macrovascular thrombi. COVID-19 is increasingly recognized as a thromboinflammatory disease where alterations of both coagulation and platelets are closely linked to mortality and clinical outcomes. Although platelets are most well known as central mediators of hemostasis, they possess chemotactic molecules, cytokines, and adhesion molecules that are now appreciated as playing an important role in the regulation of immune response. This review summarizes the current knowledge of platelet alterations observed in the context of COVID-19 and their impact upon disease pathobiology.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-12-08T00:00:00+0100
  DOI: 10.1055/a-1646-3392
Venous Thrombosis and SARS-CoV-2
- Authors: Zdanyte; Monika, Rath, Dominik, Gawaz, Meinrad, Geisler, Tobias
  Abstract: SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) infection is associated with high risk of venous and arterial thrombosis. Thrombotic complications, especially pulmonary embolism, lead to increased all-cause mortality in both intensive care unit and noncritically ill patients. Damage and activation of vascular endothelium, platelet activation, followed by thrombotic and fibrinolytic imbalance as well as hypercoagulability are the key pathomechanisms in immunothrombosis leading to a significant increase in thromboembolism in coronavirus disease 2019 (COVID-19) compared with other acute illnesses. In this review article, we discuss the incidence and prognosis, diagnosis, prevention, and treatment of venous thromboembolism in patients with COVID-19 disease, based on clinical experience and research available to date.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-12-08T00:00:00+0100
  DOI: 10.1055/a-1661-0283
Increasing Use of Anticoagulants in Germany and Its Impact on
Hospitalization for Tooth Extraction
- Authors: von Beckerath; Olga, Kröger, Knut, Santosa, Frans, Nasef, Ayat, Kowall, Bernd, Stang, Andreas
  Abstract: Objectives This article aimed to compare nationwide time trends of oral anticoagulant prescriptions with the time trend of hospitalization for tooth extraction (TE) in Germany from 2006 through 2017. Patients and Methods We derived the annual number of hospital admissions for TE from the Nationwide Hospital Referral File of the Federal Bureau of Statistics and defined daily doses (DDD) of prescribed anticoagulants in outpatients from reports of the drug information system of the statutory health insurance. Results From 2005 to 2017, annual oral anticoagulation (OAC) treatment rates increased by 143.7%. In 2017, direct oral anticoagulants (DOACs) represented 57.1% of all OAC treatments. The number of cases hospitalized for TE increased by 28.0 only. From all the cases hospitalized for TE in Germany in 2006, 14.2% had a documented history of long-term use of OACs. This proportion increased to 19.6% in 2017. Age-standardized hospitalization rates for all TE cases with long-term use of OACs increased from 6.6 in 2006 to 10.5 cases per 100,000 person-years in 2014 and remained almost unchanged thereafter. Conclusion Our comparison showed that the large increase in OAC treatment rates in general from 2006 to 2017 had only a small impact on hospitalized TE cases with long-term use of OAC which flattens since 2014.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-11-23T00:00:00+0100
  DOI: 10.1055/a-1528-0513
Non–Vitamin K Antagonist Oral Anticoagulants in Coronary Artery
Disease
- Authors: Al Said; Samer, Ellscheid, Michael, Beltsios, Eleftherios T., Frey, Norbert
  Abstract: The prevention of atherothrombotic events is the primary goal in the treatment of patients with arteriosclerotic disorders. Despite recent improvements in the management of coronary artery disease (CAD) with revascularization techniques and antiplatelet therapy, some patients remain at risk of recurrent cardiovascular events. This could be related to additional thrombin generation. As a result, there has been interest in developing novel therapies to prevent thromboembolic events, targeting thrombin-mediated pathways. These include non–vitamin K antagonist oral anticoagulants (NOACs). This article aims to summarize the recent clinical studies that investigated the role of NOACs in CAD.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-10-18T00:00:00+01:00
  DOI: 10.1055/a-1606-7523
COVID-19 as a Potential Trigger for Immune Thrombotic Thrombocytopenic
Purpura and Reason for an Unusual Treatment: A Case Report
- Authors: Schwaegermann; Marie-Kristin, Hobohm, Lukas, Rausch, Johanna, Reuter, Michael, Griemert, Thomas-Friedrich, Sivanathan, Visvakanth, Falter, Tanja, Sprinzl, Martin F., Lackner, Karl J., Galle, Peter R., Konstantinides, Stavros, Theobald, Matthias, von Auer, Charis
  Abstract: Immune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib–IX–V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-07-29T00:00:00+01:00
  DOI: 10.1055/a-1497-1054
Celiac Artery Thrombosis and Splenic Infarction as a Consequence of Mild
COVID-19 Infection: Report of an Unusual Case
- Authors: Arslan; Gokhan
  Abstract: COVID-19 has been associated with the hypercoagulable state in the literature. Patients who are admitted to the hospital with severe COVID-19 may have some thrombotic complications. These patients have a high risk for venous and arterial thrombosis of large and small vessels. Here, a 42-year-old female with celiac artery thrombosis and splenic infarction after a history of mild COVID-19 was presented.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-07-01T00:00:00+01:00
  DOI: 10.1055/a-1508-7388
Thrombosis and Dissection of the Abdominal Arteries Associated with
Infarcts of Solid Organs in a Patient with COVID-19: A Novel Clinical
Entity
- Authors: Voci; Davide, Micieli, Evy, Johner, Fabian A., Kucher, Nils, Barco, Stefano
  Abstract: The clinical spectrum of patients with coronavirus disease 2019 (COVID-19) ranges from asymptomatic cases to severe pneumonia with acute respiratory distress syndrome. COVID-19 is associated with an increased risk of thromboembolic complications, notably pulmonary embolism and deep vein thrombosis. Arterial cardiovascular complications and myocarditis have also been described in association with COVID-19, but appear to be less prevalent. In this report of a 57-year-old man with multiple splanchnic infarctions, arterial dissections and COVID-19 as the sole potential trigger, we describe a novel type of complications and put it in the context of a growing literature on this topic.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-06-22T00:00:00+01:00
  DOI: 10.1055/a-1476-7907
Effect of DDAVP on Platelet Activation and Platelet-Derived Microparticle
Generation
- Authors: Persyn; Matthieu, Athanase, Nicolas, Trossaërt, Marc, Sigaud, Marianne, Ternisien, Catherine, Béné, Marie C., Fouassier, Marc
  Abstract: Background The way by which 1-deamino-8-D-arginine vasopressin (DDAVP) acts on platelets remains unclear. Data from the literature tend to show that there is no definite effect on platelet activation, but recent work has suggested that a subtype of platelets, activated by the combined action of collagen and thrombin, was triggered by DDAVP. Moreover, platelet microparticles (PMPs), which have been shown to be procoagulant, have rarely been studied in this context. The goal of this study was to analyze the effects of DDAVP on PMPs' release through platelet activation. Methods Fifteen out of 18 consecutive patients undergoing a therapeutic test with DDAVP were included. They were suffering from factor VIII deficiency or from von Willebrand disease. The expression of P-selectin and PAC-1 binding on platelets and the numbers of circulating PMPs were evaluated ex vivo before and after DDAVP infusion. Peripheral blood was collected on CTAD to limit artifactual platelet activation. Results DDAVP induced a significant decrease of platelet counts and volume. Only small changes of P-selectin expression and PAC-1 binding were observed. Considering PMPs, two populations of patients could be defined, respectively, with (120%, n = 6) or without (21%, n = 7) an increase of PMPs after DDAVP. The decrease in platelet counts and volume remained significant in the group of responders. Conclusion This study shows that DDAVP induces the generation/release of PMPs in some patients with factor VIII deficiency and von Willebrand disease 1 hour after DDAVP infusion.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-06-17T00:00:00+01:00
  DOI: 10.1055/a-1476-7768
Association of Thrombin-Activatable Fibrinolysis Inhibitor with Acute
Pulmonary Embolism
- Authors: Yıldız; Abdulkerim, Katar, Didem, Soydaş, Ayşe Özden, Albayrak, Murat
  Abstract: Background Thrombin-activatable fibrinolysis inhibitor (TAFI) inhibits fibrinolysis and high levels may have an association with thrombosis. The aim of the current study was to investigate the association of TAFI antigen levels with pulmonary thromboembolism (PTE). Patients and Methods A case–control study was conducted with 29 patients with PTE and 17 age- and gender-matched control individuals. Plasma levels of TAFI were measured at the time of diagnosis, then at 3 and 6 months after the event. Results Initial TAFI levels (%) were higher in patients with PTE than in the control group Initial TAFI levels (%) were higher in patients with PTE than in the control group (190,0 [65,0–250,0] vs 133,0 [83,0–153,0]; p = 0.003). TAFI levels significantly decreased at the third and sixth months after initial diagnosis (p 0.05). In the sixth month of treatment, patients with residual thrombosis were seen to have similar baseline levels and reductions of TAFI as patients without residual thrombosis (p > 0.05). Conclusion The result of this study suggests that high TAFI levels may have a role in the occurrence of PTE without impact on treatment outcome.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-06-17T00:00:00+01:00
  DOI: 10.1055/a-1411-7807
Spontaneous Intra-abdominal Hemorrhage in a Hemophilia A Patient
Masquerading as Intra-abdominal Pseudotumor
- Authors: Jayant; Divij, Sakaray, Yashwant, Thakur, Uttam, Kaman, Lileshwar, Jain, Arihant
  Abstract: Spontaneous intra-abdominal hemorrhage is very rare in hemophilia A patients. High index of suspicion is necessary for successful management. Surgical intervention may be required in cases of diagnostic uncertainty and for adequate hemostasis. Here we report a case of spontaneous hemorrhage in the peritoneal cavity in hemophilia A patient, who was managed with surgery and factor VIII supplementation. Patient did well in the postoperative periods.
  Citation: Hamostaseologie ; : -
  PubDate: 2021-04-15T00:00:00+01:00
  DOI: 10.1055/a-1384-3696