Insights from the Hereditary Thrombotic Thrombocytopenic Purpura Registry: Discussion of Key Findings Based on Individual Cases from Switzerland.
Discussion of Key Findings Based on Individual Cases from Switzerland. Hamostaseologie. 2020 Nov;40(S 01):S5-S14 Authors: Kremer Hovinga JA, Braschler TR, Buchkremer F, Farese S, Hengartner H, Lovey PY, Largiadèr CR, Mansouri Taleghani B, Tarasco E Abstract The Hereditary TTP Registry is an international cohort study for patients with a confirmed or suspected diagnosis of hereditary thrombotic thrombocytopenic purpura (hTTP) and their family members. Hereditary TTP is an ultra-rare blood disorder (prevalence of ∼1-2 cases per million), the result of autosomal-recessively inherited congenita...
Source: Hamostaseologie - November 1, 2020 Category: Hematology Authors: Kremer Hovinga JA, Braschler TR, Buchkremer F, Farese S, Hengartner H, Lovey PY, Largiadèr CR, Mansouri Taleghani B, Tarasco E Tags: Hamostaseologie Source Type: research

Molecular Profiling of Liver Sinusoidal Endothelial Cells in Comparison to Hepatocytes: Reflection on Which Cell Type Should Be the Target for Gene Therapy.
Abstract Human factor VIII (FVIII), which deficiency leads to hemophilia A, is largely synthesized and secreted by the liver sinusoidal endothelial cells (LSECs). However, the characteristics of these cells that secrete FVIII are not well known. We have previously reported that based on genome-wide expression and CpG methylation profiling, LSECs have a distinct molecular profile that distinguishes them from other endothelial cells. Hepatocytes are targeted by gene therapy protocols to treat hemophilia A. However, the hepatocyte is not the natural site for FVIII synthesis and current gene therapy protocols are elic...
Source: Hamostaseologie - November 1, 2020 Category: Hematology Authors: El-Maarri O, Jamil MA, Oldenburg J Tags: Hamostaseologie Source Type: research

Extended Half-Life Factor VIII/Factor IX Products: Assay Discrepancies and Implications for Hemophilia Management.
nburg J Abstract Due to structural differences between extended half-life (EHL) factor VIII (FVIII) or FIX products and equivalent plasma wild-type molecules used for assay calibration, reagent-dependent discrepancies during monitoring of FVIII- and FIX-replacement therapies with EHL products have been described. To assess the performance of available one-stage clotting and chromogenic substrate assays on the Siemens Atellica COAG 360 analyzer, an in vitro study using spiked plasma samples was performed. The described results confirm previously described findings and allowed allocation of each EHL product to an ap...
Source: Hamostaseologie - November 1, 2020 Category: Hematology Authors: Müller J, Goldmann G, Marquardt N, Pötzsch B, Oldenburg J Tags: Hamostaseologie Source Type: research

Flow Cytometric Assessment of AKT Signaling in Platelet Activation: An Alternative Diagnostic Tool for Small Volumes of Blood.
CONCLUSION:  An activation of the AKT-signal pathway by different agonists can clearly be detected on the flow cytometer, indicating that the visualization of signaling in platelets by flow cytometry might be an efficient alternative for light transmission aggregometry to test platelet function in children. PMID: 33187007 [PubMed - in process] (Source: Hamostaseologie)
Source: Hamostaseologie - November 1, 2020 Category: Hematology Authors: Althaus K, Wagner M, Marini I, Bakchoul T, Pelzl L Tags: Hamostaseologie Source Type: research

A Collection of Jewels.
Abstract The authors of this issue of Hämostaseologie-Progress in Haemostasis on "PROGRESS AND PITFALLS IN LABORATORY HEMOSTASIS DIAGNOSIS: " were asked to write conceptual reviews, enucleating the quintessence of their subjects in order to share it with a wide audience. All performed a masterful job and you can now enjoy a collection of jewels, each with its peculiar character, which are presented in five "invisible" sections. PMID: 33091946 [PubMed - in process] (Source: Hamostaseologie)
Source: Hamostaseologie - October 24, 2020 Category: Hematology Authors: Alberio L Tags: Hamostaseologie Source Type: research

Hemostasis Laboratory Diagnostics: Characteristics, Communication Issues, and Current Challenges Resulting from Centralization of Laboratory Medicine.
Abstract Laboratory diagnostics of patients with bleeding and thrombotic disorders can be a delicate task, which requires special skills and expertise. In this article, characteristic features of hemostasis testing are reviewed, including staged protocols and synoptic assessment of the patient history, clinical symptoms, and laboratory findings. Despite major progress in the diagnostic and therapeutic management, centralized testing of hemostasis can be associated with substantial challenges, resulting from the current dissociation between the clinical and laboratory world. To address some of these challenges, pos...
Source: Hamostaseologie - October 24, 2020 Category: Hematology Authors: Scharf RE Tags: Hamostaseologie Source Type: research

Challenges in Detecting Clinically Relevant Heparin-Induced Thrombocytopenia Antibodies.
Abstract Heparin-induced thrombocytopenia (HIT) is an antibody-mediated hypercoagulable state featuring high thrombosis risk and distinct pathogenesis involving immunoglobulin G-mediated platelet activation. The target of the immune response is a cationic "self" protein, platelet factor 4 (PF4), rendered antigenic by heparin. A key problem is that only a minority of anti-PF4/polyanion antibodies induced by heparin are pathogenic, i.e., capable of causing platelet activation and thereby clinical HIT. Since thrombocytopenia occurs frequently in hospitalized, heparin-treated patients, testing for "HIT ...
Source: Hamostaseologie - October 24, 2020 Category: Hematology Authors: Warkentin TE Tags: Hamostaseologie Source Type: research

Massive Hemorrhage: The Role of Whole Blood Viscoelastic Assays.
Abstract Viscoelastic whole blood tests are increasingly used to guide hemostatic therapy in bleeding patients in the perioperative, trauma, and obstetric settings. Compared with standard laboratory tests of hemostasis, they have a shorter turnaround time and provide simultaneous information on various aspects of clot formation and lysis. The two available brands TEG (thromboelastography) and ROTEM (rotational thromboelastometry) provide devices that are either manually operated or fully automated. The automation allows for the assays to be used as point-of-care tests increasing their usefulness in massively bleed...
Source: Hamostaseologie - October 24, 2020 Category: Hematology Authors: Scala E, Marcucci C Tags: Hamostaseologie Source Type: research

Stipendien, F örderpreise und neue Early Career Research Grants der GTH: Bewerbungsdeadline 30. November 2020.
Stipendien, Förderpreise und neue Early Career Research Grants der GTH: Bewerbungsdeadline 30. November 2020. Hamostaseologie. 2020 Nov;40(4):537-542 Authors: PMID: 33091950 [PubMed - in process] (Source: Hamostaseologie)
Source: Hamostaseologie - October 24, 2020 Category: Hematology Tags: Hamostaseologie Source Type: research

§132i-Verträge: Aktueller Stand (Stellungnahme: PD Dr. Koscielny). Aktuelle Kritik zu Bestrebungen der Digitalisierung im Gesundheitswesen (Stellungnahme: PD Dr. Sucker).
§132i-Verträge: Aktueller Stand (Stellungnahme: PD Dr. Koscielny). Aktuelle Kritik zu Bestrebungen der Digitalisierung im Gesundheitswesen (Stellungnahme: PD Dr. Sucker). Hamostaseologie. 2020 Nov;40(4):543-545 Authors: Koscielny J, Sucker C, Kappert G PMID: 33091951 [PubMed - in process] (Source: Hamostaseologie)
Source: Hamostaseologie - October 24, 2020 Category: Hematology Authors: Koscielny J, Sucker C, Kappert G Tags: Hamostaseologie Source Type: research

Evaluation of the U.S. Adherence Questionnaires VERITAS-PRO and VERITAS-PRN for Use in Patients with Hemophilia in the German Healthcare System.
CONCLUSION:  VERITAS-PRO and VERITAS-PRN have only very limited benefits for the German healthcare system. Since nonadherence has a great impact on the morbidity of PWH and on the costs for the healthcare system, there is a need for adherence instruments that are adapted to the specific needs of PWH in the German healthcare system. PMID: 33086407 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - October 21, 2020 Category: Hematology Authors: von Mackensen S, Douma Y, Halimeh S Tags: Hamostaseologie Source Type: research

[Clinical skills].
PMID: 33064178 [PubMed - in process] (Source: Hamostaseologie)
Source: Hamostaseologie - October 1, 2020 Category: Hematology Authors: Buerke M, Hermes C, Petros S Tags: Med Klin Intensivmed Notfmed Source Type: research

How to Capture the Bleeding Phenotype in FXI-Deficient Patients.
Abstract Factor XI (FXI) is a serine protease involved in the propagation phase of coagulation and in providing clot stability. Several mutations in the F11 gene lead to FXI deficiency, a rare mild bleeding disorder. Current laboratory methods are unable to assess bleeding risk in FXI-deficient patients, because the degree of bleeding tendency does not correlate with plasma FXI activity as measured by routine coagulometric aPTT-based assays. Bleeding manifestations are highly variable among FXI-deficient patients and FXI replacement therapy can be associated with an increased thrombotic risk. A correct evaluation ...
Source: Hamostaseologie - September 30, 2020 Category: Hematology Authors: Bertaggia Calderara D, Zermatten MG, Aliotta A, Alberio L Tags: Hamostaseologie Source Type: research

Translating Laboratory Tests into Clinical Practice: A Conceptual Framework.
Abstract The use of laboratory biomarkers in clinical practice is rapidly increasing. Laboratory tests are, however, rarely evaluated adequately before implementation, and the utility of many tests is essentially unclear. An important reason for this knowledge gap is that a comprehensive and generally accepted methodological framework supporting evaluation studies is essentially lacking. Researchers, clinicians, and decision-makers are often not aware of the methodological tools available and face problems with the appraisal of a test's utility. With the present article, I aim to summarize current concepts and met...
Source: Hamostaseologie - September 21, 2020 Category: Hematology Authors: Nagler M Tags: Hamostaseologie Source Type: research

The Art of Detecting Antibodies against Factor VIII.
This article reviews the available assays and their clinical interpretation in patients with congenital and acquired hemophilia. PMID: 32961576 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - September 21, 2020 Category: Hematology Authors: Tiede A, Alberio L Tags: Hamostaseologie Source Type: research

Antiphospholipid Antibodies Are Not Associated with Clinical Parameters or Prognostic Outcomes in Patients with Non-Hodgkin's Lymphoma.
CONCLUSION:  APAs are encountered more frequently in NHL patients than in the general population. However, APAs do not correlate with disease severity, thrombosis risk, treatment outcome, or overall survival. PMID: 32932545 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - September 14, 2020 Category: Hematology Authors: Pal R, Varma S, Ahluwalia J, Prakash G Tags: Hamostaseologie Source Type: research

Laboratory Techniques Used to Diagnose Constitutional Platelet Dysfunction.
Abstract Platelets play a major role in primary hemostasis, where activated platelets form plugs to stop hemorrhaging in response to vessel injuries. Defects in any step of the platelet activation process can cause a variety of platelet dysfunction conditions associated with bleeding. To make an accurate diagnosis, constitutional platelet dysfunction (CPDF) should be considered once von Willebrand disease and drug intake are ruled out. CPDF may be associated with thrombocytopenia or a genetic syndrome. CPDF diagnosis is complex, as no single test enables the analysis of all aspects of platelet function. Furthermor...
Source: Hamostaseologie - September 14, 2020 Category: Hematology Authors: Ibrahim-Kosta M, Alessi MC, Hezard N Tags: Hamostaseologie Source Type: research

What about Platelet Function in Platelet Concentrates?
Abstract The characterization of platelet concentrates (PCs) in transfusion medicine has been performed with different analytical methods and platelet lesions (from biochemistry to cell biology) have been documented. In routine quality assessment and validation of manufacturing processes of PCs for transfusion purposes, only basic parameters are monitored and the platelet functions are not included. However, PCs undergo several manipulations during the processing and the basic parameters do not provide sensitive analyses to properly picture out the impact of the blood component preparation and storage on platelets...
Source: Hamostaseologie - September 14, 2020 Category: Hematology Authors: Prudent M Tags: Hamostaseologie Source Type: research

Impact of complete surgical resection on outcome in aggressive non-Hodgkin lymphoma treated with immunochemotherapy.
CONCLUSION: Young patients with early stage DLBCL may benefit from complete lymphoma resection prior to immunochemotherapy. PMID: 32926763 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - September 13, 2020 Category: Hematology Authors: Schmitz C, Rekowski J, Müller SP, Farsijani N, Hertenstein B, Franzius C, von Verschuer U, La Rosée P, Freesmeyer M, Wilop S, Krohn T, Raghavachar A, Ganser A, Bengel FM, Prange-Krex G, Kroschinsky F, Kotzerke J, Giagounidis A, Dührsen U, Hüttmann A Tags: Cancer Med Source Type: research

Double Heterozygous Mutations (Cys247Tyr and 252delAsn) Cause Factor XII Deficiency in a Chinese Family.
CONCLUSION:  The c.797G > A heterozygous missense variation and the c.809_811delACA heterozygous deletion variation are associated with decreased FXII levels in this family, of which c.797G > A is first reported in the world. PMID: 32916752 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - September 10, 2020 Category: Hematology Authors: Wang Y, Zhang H, Liu S, Ye J Tags: Hamostaseologie Source Type: research

In Silico Hemostasis Modeling and Prediction.
Abstract Computational physiology, i.e., reproduction of physiological (and, by extension, pathophysiological) processes in silico, could be considered one of the major goals in computational biology. One might use computers to simulate molecular interactions, enzyme kinetics, gene expression, or whole networks of biochemical reactions, but it is (patho)physiological meaning that is usually the meaningful goal of the research even when a single enzyme is its subject. Although exponential rise in the use of computational and mathematical models in the field of hemostasis and thrombosis began in the 1980s (first for...
Source: Hamostaseologie - September 10, 2020 Category: Hematology Authors: Nechipurenko DY, Shibeko AM, Sveshnikova AN, Panteleev MA Tags: Hamostaseologie Source Type: research

Critical Bleeding in Acquired Hemophilia A: Bypassing Agents or Recombinant Porcine Factor VIII?
Abstract Acquired hemophilia A (AHA) is caused by autoantibodies neutralizing coagulation factor VIII (FVIII). In the presence of inhibitors against FVIII, acute bleeds can be managed with bypassing agents, including recombinant factor VIIa (eptacog alfa activated, NovoSeven) and activated prothrombin complex concentrate (FEIBA), as well as recombinant porcine FVIII (susoctocog alfa, Obizur). Studies comparing these agents directly are not available, and indirect evidence suggests an overall similar efficacy. Selecting an agent in clinical practice therefore depends on (1) availability of agent, (2) safety profile...
Source: Hamostaseologie - September 10, 2020 Category: Hematology Authors: Tiede A Tags: Hamostaseologie Source Type: research

COVID-19 Coagulopathy with Superior Mesenteric Vein Thrombosis Complicated by an Ischaemic Bowel.
We describe a patient with COVID-19 who developed an acute superior mesenteric vein thrombosis with acute intestinal obstruction despite adequate anticoagulation. PMID: 32894875 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - September 6, 2020 Category: Hematology Authors: Fan BE, Chang CCR, Teo CHY, Yap ES Tags: Hamostaseologie Source Type: research

Trauma-Induced Coagulopathy and Massive Bleeding: Current Hemostatic Concepts and Treatment Strategies.
l;chl H Abstract Hemorrhage after trauma remains a significant cause of preventable death. Trauma-induced coagulopathy (TIC) at the time of hospital admission is associated with an impaired outcome. Rather than a universal phenotype, TIC represents a complex hemostatic disorder, and standard coagulation tests are not designed to adequately reflect the complexity of TIC. Viscoelastic testing (VET) has gained increasing interest for the characterization of TIC because it provides a more comprehensive depiction of the coagulation process. Thus, VET has been established as a point-of-care-available hemostatic monitori...
Source: Hamostaseologie - September 6, 2020 Category: Hematology Authors: Gratz J, Oberladstätter D, Schöchl H Tags: Hamostaseologie Source Type: research

[Laboratory testing in intensive care medicine].
Abstract Despite the tremendous technological developments in medicine, careful history-taking and clinical examination remain the cornerstones of diagnostics. Numerous laboratory tests are ordered in intensive care and emergency medicine. The rate of overutilization of these tests during initial patient admission is almost 50%. Patient history may be frequently insufficient for conducting targeted laboratory testing, and concern about not overlooking a pathology also contributes to laboratory test overutilization. On the other hand, laboratory test profiles are frequently defined a priori to simplify th...
Source: Hamostaseologie - September 2, 2020 Category: Hematology Authors: Petros S, Weidhase L Tags: Med Klin Intensivmed Notfmed Source Type: research

Laboratory Assessment of Coagulation Factor XIII.
Abstract Laboratory diagnosis of congenital and acquired deficiencies of coagulation factor XIII (FXIII) can be challenging. Determination of FXIII function requires specific and sensitive assays which are not always available. This brief review article summarizes currently used FXIII assay methods, their principles and difficulties, and discusses the recommended diagnostic workup in case of a suspected FXIII deficiency. The article also briefly touches on experimental methods used in FXIII research. PMID: 32869231 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - August 30, 2020 Category: Hematology Authors: Schroeder V Tags: Hamostaseologie Source Type: research

Clinical Problem Solving and Using New Paths in the Laboratory: Learning from Case Studies.
e B Abstract Three selected casuistic studies of individual patient problems from my long-standing professional experience are presented. I intend to highlight the potential contribution of case studies, including new approaches of laboratory investigation, for the advancement of pathophysiological knowledge and would encourage to "rehabilitate" the low academic profile generally attributed to "case reports." PMID: 32820490 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - August 19, 2020 Category: Hematology Authors: Lämmle B Tags: Hamostaseologie Source Type: research

Productivity of Young Experts in Hemostasis and Thrombosis before and during the SARS-CoV-2 Pandemic-A Special Issue.
PMID: 32726820 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Scharf RE Tags: Hamostaseologie Source Type: research

The Millennial Generation of Thrombosis and Hemostasis.
PMID: 32726821 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Barco S Tags: Hamostaseologie Source Type: research

Screening for Occult Cancer in Patients with Venous Thromboembolism: Past, Present, and Future.
Abstract A strong link between cancer and thrombosis has been well recognized. The occurrence of venous thromboembolism (VTE) can be the first clinical sign of an undiagnosed (i.e., occult) cancer. Cancer is more often diagnosed after unprovoked compared with provoked VTE events, with a reported risk in recent studies of around 5%. Extensive, imaging-based screening strategies to detect occult cancer after unprovoked VTE do not appear to have a clear clinical benefit compared with a more limited cancer screening. To identify patients with unprovoked VTE at high risk of occult cancer, risk factors have been explore...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: van Es N, Ay C, Jara-Palomares L Tags: Hamostaseologie Source Type: research

How to Differentiate Recurrent Deep Vein Thrombosis from Postthrombotic Syndrome.
Abstract Venous thromboembolism (VTE) is associated with significant morbidity and mortality. Accuracy of diagnosis is thus of vital importance. Failure to diagnose VTE increases the risk of progression and complications. Conversely, anticoagulation as a result of an incorrect diagnosis exposes patients to the associated hazards of bleeding. The diagnostic management of recurrent deep vein thrombosis (DVT) and postthrombotic syndrome (PTS) is especially challenging due to the lack of well-established diagnostic standards. Particularly, the differentiation between the two is notoriously difficult as symptoms, clini...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Gautam G, Sebastian T, Klok FA Tags: Hamostaseologie Source Type: research

Management of Anticoagulant Treatment and Anticoagulation-Related Complications in Nonagenarians.
Abstract Given the aging population, the burden of age-dependent diseases is growing. Despite this, elderly patients are often underrepresented in clinical trials and little data are available on current anticoagulant management and outcomes in this unique population, especially those aged 90 years or older. There is uncertainty, and a fear of "doing harm," that often leads to de-prescription of antithrombotic agents in nonagenarian patients. Decision-making concerning the use of anticoagulant treatment needs to balance the risk of thrombotic events against the risk of major bleeding, especially intracra...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Giustozzi M, Castellucci LA, Barnes GD Tags: Hamostaseologie Source Type: research

Head, Neck, and Abdominopelvic Septic Thrombophlebitis: Current Evidence and Challenges in Diagnosis and Treatment.
Abstract Septic thrombophlebitis (STP) is a complex, cross-disciplinary clinical condition that combines a localized infection with a neighboring venous thrombosis. STP can occur at several possible anatomic sites, such as dural sinuses, jugular vein (Lemierre syndrome), portal vein (pylephlebitis), and pelvic veins. Its high mortality in the preantibiotic era improved considerably with the introduction of modern antibiotics. However, little evidence exists to date to guide its clinical management. The incidence of STP or its risk factors may be increasing, and its mortality may still be considerable. These trends...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Valerio L, Riva N Tags: Hamostaseologie Source Type: research

The Evolution of Hemophilia Care: Clinical and Laboratory Advances, Opportunities, and Challenges.
Abstract Hemophilia A (HA) and B (HB) are X-linked bleeding disorders caused by mutations in the F8 or F9 gene that result in the absence, or reduced activity, of the corresponding clotting factor. The severity of bleeding and related complications is proportional to the amount of residual circulating functional factor. The development of a safe and effective hemophilia treatment lasted several decades and has been mainly based on clotting factor replacement. Advances in the engineering and manufacturing of clotting concentrates have led to the widespread availability of extended half-life products that reduced th...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Trinchero A, Sholzberg M, Matino D Tags: Hamostaseologie Source Type: research

Current and Future Perspectives on ADAMTS13 and Thrombotic Thrombocytopenic Purpura.
Abstract Thrombotic thrombocytopenic purpura (TTP) is a rare, relapsing, and life-threatening disorder with an annual incidence of 10 cases per million people. TTP is a thrombotic microangiopathy characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia. The disease is caused by a severe deficiency of the enzyme ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13), which can either be acquired, mainly by autoantibodies targeting ADAMTS13, or congenital due to mutations in the ADAMTS13 gene. Thanks to the establishment of national registrie...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Roose E, Joly BS Tags: Hamostaseologie Source Type: research

Role of Platelet Cytoskeleton in Platelet Biomechanics: Current and Emerging Methodologies and Their Potential Relevance for the Investigation of Inherited Platelet Disorders.
Abstract Cytoskeleton is composed of more than 100 proteins and represents a dynamic network of the cellular cytoplasm. Cytoskeletal functions include spatial organization of cellular components, structural connection of the cell with external environment, and biomechanical force generation. Cytoskeleton takes part, at different levels, in all phases of platelet biogenesis: megakaryocyte (MK) differentiation, MK maturation, and platelet formation. In addition, it also plays a major role in each stage of platelet function. Inherited platelet disorders (IPDs) are a group of rare diseases featured by low platelet cou...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Zaninetti C, Sachs L, Palankar R Tags: Hamostaseologie Source Type: research

Use of Anticoagulants in Patients with Pulmonary Hypertension.
Abstract Since the earliest works on the understanding of different forms of pulmonary hypertension, thrombosis has been involved in the pathophysiology of the disease, both in pulmonary arterial hypertension (PAH) and in chronic thromboembolic pulmonary hypertension (CTEPH). Autopsy and then pathophysiological data paved the way for the use of anticoagulants as a treatment for PAH and CTEPH. In PAH their role has diminished with the advent of specific targeted therapies, but they are still prescribed in more than half of PAH patients, because of concomitant venous thromboembolism or atrial fibrillation. In CTEPH ...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Bertoletti L, Mismetti V, Giannakoulas G Tags: Hamostaseologie Source Type: research

Understanding and Preventing Placenta-Mediated Pregnancy Complications.
cute;inle F Abstract Placenta-mediated pregnancy complications are clinically important conditions and include preeclampsia, placental abruption, intrauterine growth restriction, and late fetal loss. Pathophysiology is complex, and may be linked to prothrombotic disorders such as antiphospholipid syndrome, whose understanding is still evolving. In this narrative review, we will present the latest evidence to better understand hemostatic mechanisms of preeclampsia, as well as in women with placenta-mediated pregnancy complications and inherited thrombophilia or antiphospholipid antibodies. Using four clinical scena...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Skeith L, Blondon M, Ní Áinle F Tags: Hamostaseologie Source Type: research

Can We Measure the Individual Prothrombotic or Prohemorrhagic Tendency by Global Coagulation Tests?
l H Abstract Hemostasis is a complex process in which abnormalities can cause shifts toward prothrombotic or prohemorrhagic states resulting in thrombosis or bleeding, respectively. Several coagulation tests may be required to characterize these defects but may yet not always reflect a patient's true hemostatic capacity. Thus, global coagulation tests aiming to simulate the coagulation process in vitro instead of measuring single components thereof are certainly of interest to assess prothrombotic or prohemorrhagic tendencies. This review describes the development and application of global coagulation tests, conce...
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Reda S, Morimont L, Douxfils J, Rühl H Tags: Hamostaseologie Source Type: research

About mentees and mentors: what kind of advanced training should young scientists receive? - A personal reflection.
Abstract Comprehensive training of talented MD or PhD students and postdoctoral fellows is one of the essential requirements for a future career as an established investigator or even an outstanding scientist. Here, from personal experience, several relevant aspects are summarized including the high responsibility of the academic mentor. Specifically, the interaction of the mentor with fellows, the choice of the research project, and the impact of the mentor's personality on mentees are discussed. PMID: 32726832 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Scharf RE Tags: Hamostaseologie Source Type: research

Erfolgreicher Launch der GTH Mediathek im Juni.
Authors: PMID: 32726833 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2020 Category: Hematology Tags: Hamostaseologie Source Type: research

EBM-Reform zum 01.04.2020.
PMID: 32726834 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 30, 2020 Category: Hematology Authors: Sucker C, Kappert G, Koscielny J Tags: Hamostaseologie Source Type: research

In Vitro Closure Times (PFA-100) Are Different Between Peritoneal Dialysis and Hemodialysis.
a M Abstract INTRODUCTION:  Platelet dysfunction is not uncommon in patients with end-stage renal disease (ESRD). Type of renal replacement therapy may have an effect on platelet functions, which has not been well investigated. We evaluated in vitro closure time (CT) differences between peritoneal dialysis (PD) and hemodialysis (HD) patients using platelet function analyzer (PFA-100)and observed a significant difference between these renal replacement therapies. METHODS:  Patients with ESRD undergoing PD (n = 24) or HD (n = 23) for more than 6 months were included. Blood ...
Source: Hamostaseologie - July 26, 2020 Category: Hematology Authors: Gokalp C, Karadag FK, Braunisch MC, Schmaderer C, Gunay E, Kiper HD, Tobu M, Ustün C, Demirci MS, Ozkahya M Tags: Hamostaseologie Source Type: research

Origins, Development, Current Challenges and Future Directions with Activated Prothrombin Complex Concentrate for the Treatment of Patients with Congenital Haemophilia with Inhibitors.
ier C Abstract Congenital haemophilia A (HA) is caused by deficiency of coagulation factor VIII (FVIII) activity, leading to spontaneous or traumatic bleeding events. While FVIII replacement therapy can treat and prevent bleeds, approximately 30% of patients with severe HA develop inhibitor antibodies that render FVIII replacement therapy ineffective. The bypassing agents (BPAs), activated prothrombin complex concentrate (aPCC) and recombinant activated FVII, first approved in 1977 and 1996, respectively, act to generate thrombin independent of pathways that involve factors IX and VIII. Both may be used in patient...
Source: Hamostaseologie - July 26, 2020 Category: Hematology Authors: Brackmann HH, Schramm W, Oldenburg J, Cano V, Turecek PL, Négrier C Tags: Hamostaseologie Source Type: research

Correction: Frequency of infections in 948 MPN patients: a prospective multicenter patient-reported pilot study.
idel FH Abstract An amendment to this paper has been published and can be accessed via a link at the top of the paper. PMID: 32651539 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - July 9, 2020 Category: Hematology Authors: Crodel CC, Jentsch-Ullrich K, Koschmieder S, Kämpfe D, Griesshammer M, Döhner K, Jost PJ, Wolleschak D, Isfort S, Stegelmann F, Jilg S, Hofmann V, Auteri G, Rachow T, Ernst P, Brioli A, von Lilienfeld-Toal M, Hochhaus A, Palandri F, Heidel FH Tags: Leukemia Source Type: research

From Routine to Research Laboratory: Strategies for the Diagnosis of Congenital Fibrinogen Disorders.
Abstract Congenital fibrinogen disorders (CFDs) encompass a heterogeneous group of fibrinogen defects with a wide spectrum of biological and clinical features. An accurate diagnosis is thus essential to assure the optimal management for the patient. Diagnosis involves a multistep approach starting with routine coagulation assays and assessment of functional and antigenic fibrinogen followed by identification of the molecular anomaly. However, the diagnosis of CFD can be challenging as the sensitivity and specificity of coagulation assays depend on the fibrinogen level as well as on the fibrinogen variant. In addit...
Source: Hamostaseologie - July 8, 2020 Category: Hematology Authors: Casini A Tags: Hamostaseologie Source Type: research

Navigating the Myriad of von Willebrand Factor Assays.
Abstract von Willebrand factor (VWF) represents a large and complex adhesive plasma protein whose main function is to provide a bridge between blood platelets and damaged endothelium, and thus facilitate primary hemostasis. VWF also binds to FVIII, preventing early proteolysis, and delivers this cargo to sites of vascular injury, thereby promoting clot formation and secondary hemostasis. An absence, deficiency, or defect in VWF can lead to a bleeding diathesis called von Willebrand disease (VWD), considered the most common inherited bleeding disorder. Contemporary laboratory assays used in VWD diagnosis/exclusion ...
Source: Hamostaseologie - June 25, 2020 Category: Hematology Authors: Favaloro EJ Tags: Hamostaseologie Source Type: research

Prevention of Venous Thromboembolism during Pregnancy and the Puerperium with a Special Focus on Women with Hereditary Thrombophilia or Prior VTE-Position Paper of the Working Group in Women's Health of the Society of Thrombosis and Haemostasis (GTH).
Abstract Venous thromboembolism (VTE) is a major cause of maternal morbidity during pregnancy and the postpartum period. Because there is a lack of adequate study data, management strategies for the prevention of VTE during pregnancy have mainly been deduced from case-control and observational studies and extrapolated from recommendations for non-pregnant patients. The decision for or against pharmacologic thromboprophylaxis must be made on an individual basis weighing the risk of VTE against the risk of adverse side effects such as severe bleeding complications. A comprehensive, multidisciplinary approach is ofte...
Source: Hamostaseologie - June 25, 2020 Category: Hematology Authors: Hart C, Bauersachs R, Scholz U, Zotz R, Bergmann F, Rott H, Linnemann B Tags: Hamostaseologie Source Type: research

Practical Guidance of the GTH Haemophilia Board on the Use of Emicizumab in Patients with Haemophilia A.
In conclusion, this study is designed to support haemophilia physicians using Emicizumab in physicians treating hemophilia and using (PWHAs). With further post-marketing experience and trials, regular updates are necessary. PMID: 32588417 [PubMed - as supplied by publisher] (Source: Hamostaseologie)
Source: Hamostaseologie - June 24, 2020 Category: Hematology Authors: Holstein K, Albisetti M, Bidlingmaier C, Halimeh S, Heine S, Klamroth R, Königs C, Kurnik K, Male C, Oldenburg J, Streif W, Wermes C, Escuriola-Ettingshausen C, ‘Ständige Kommission Hämophilie’ (Haemophilia board) of the German, Swiss Austrian Soci Tags: Hamostaseologie Source Type: research

Coagulopathy in COVID-19 and Its Implication for Safe and Efficacious Thromboprophylaxis.
Abstract The novel coronavirus, SARS-CoV-2, is causing a global pandemic of life-threatening multiorgan disease, called COVID-19. Accumulating evidence indicates that patients with COVID-19 are at significant risk of thromboembolic complications, mainly affecting the venous, but also the arterial vascular system. While the risk of venous thromboembolism (VTE) appears to be higher in patients requiring intensive care unit support compared to those admitted to general wards, recent autopsy findings and data on the timing of VTE diagnosis relative to hospitalization clearly suggest that thromboembolic events also con...
Source: Hamostaseologie - June 3, 2020 Category: Hematology Authors: Langer F, Kluge S, Klamroth R, Oldenburg J Tags: Hamostaseologie Source Type: research