HamostaseologiePub Date : 2024-04-01Epub Date: 2024-03-26DOI: 10.1055/a-2229-4190
F Joachim Meyer, Christian Opitz
{"title":"Post-Pulmonary Embolism Syndrome: An Update Based on the Revised AWMF-S2k Guideline.","authors":"F Joachim Meyer, Christian Opitz","doi":"10.1055/a-2229-4190","DOIUrl":"10.1055/a-2229-4190","url":null,"abstract":"<p><p>In survivors of acute pulmonary embolism (PE), the post-PE syndrome (PPES) may occur. In PPES, patients typically present with persisting or progressive dyspnea on exertion despite 3 months of therapeutic anticoagulation. Therefore, a structured follow-up is warranted to identify patients with chronic thromboembolic pulmonary disease (CTEPD) with normal pulmonary pressure or chronic thromboembolic pulmonary hypertension (CTEPH). Both are currently understood as a dual vasculopathy, that is, secondary arterio- and arteriolopathy, affecting the large and medium-sized pulmonary arteries as well as the peripheral vessels (diameter < 50 µm). The follow-up algorithm after acute PE commences with identification of clinical symptoms and risk factors for CTEPH. If indicated, a stepwise performance of echocardiography, ventilation-perfusion scan (or alternative imaging), N-terminal prohormone of brain natriuretic peptide (NT-proBNP) level, cardiopulmonary exercise testing, and pulmonary artery catheterization with angiography should follow. CTEPH patients should be treated in a multidisciplinary center with adequate experience in the complex therapeutic options, comprising pulmonary endarterectomy, balloon pulmonary angioplasty, and pharmacological interventions.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":"128-134"},"PeriodicalIF":3.2,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140295419","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 : 2024-04-01Epub Date: 2023-11-22DOI: 10.1055/a-2178-6508
Hanno Riess, Jan Beyer-Westendorf, Uwe Pelzer, Robert Klamroth, Birgit Linnemann
{"title":"Cancer-Associated Venous Thromboembolism-Diagnostic and Therapeutic Considerations: An Update Based on the Revised AWMF S2k Guideline.","authors":"Hanno Riess, Jan Beyer-Westendorf, Uwe Pelzer, Robert Klamroth, Birgit Linnemann","doi":"10.1055/a-2178-6508","DOIUrl":"10.1055/a-2178-6508","url":null,"abstract":"<p><p>Patients with cancer are prone to develop venous thromboembolism (VTE) with negative impact on quality of life, morbidity, and mortality. Treatment of established VTE is often complex in patients with cancer. Treatment of cancer-associated VTE (CAT) basically comprises initial and maintenance treatment, for 3 to 6 months, secondary preventions, and treatment in special situations. Therapeutic anticoagulation is the treatment of choice in CAT. In addition to the efficacy and safety of low-molecular-weight heparin (LMWH) that had been recommended for decades, direct oral anti-factor Xa inhibitors, a subgroup of direct oral anticoagulants (DOACs), demonstrated their advantages along with the accompanying concerns in several randomized controlled treatment trials of CAT. The latest guidelines, such as the German AWMF-S2k Guideline \"Diagnostics and Therapy of Venous Thrombosis and Pulmonary Embolism,\" agree with each other on most aspects with respect to the treatment of CAT. Encompassing recent clinical studies, and meta-analyses, as well as the focus on some special management aspects of CAT, the objective of this review is to present a current overview and recommendations for the treatment of CAT.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":"143-149"},"PeriodicalIF":3.2,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138296606","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 : 2024-03-22DOI: 10.1055/a-2266-7984
Adeline Blandinières, Sophie Combe, Noémie Chanson, Olivier Lambotte, Cécile Lavenu-Bombled
{"title":"Use of Vonicog Alpha and Acquired von Willebrand Syndrome, a New Approach: A Case Report.","authors":"Adeline Blandinières, Sophie Combe, Noémie Chanson, Olivier Lambotte, Cécile Lavenu-Bombled","doi":"10.1055/a-2266-7984","DOIUrl":"https://doi.org/10.1055/a-2266-7984","url":null,"abstract":"<p><p>Therapeutic management of acquired von Willebrand syndrome (AVWS) can be challenging, particularly in cases of AVWS associated with monoclonal IgM such as Waldenström macroglobulinemia (WM) where several therapeutic options may be ineffective. Here, we describe the case of an 88-year-old patient who developed AVWS during follow-up for WM. The presence of a severe bleeding symptomatology not controlled by several therapies (plasma-derived von Willebrand factor, plasmapheresis) led us to introduce a supplementation with recombinant von Willebrand factor, vonicog α (Veyvondi, Takeda, Japan), starting at a dose of 50 IU/kg/d. This supplementation allowed clinical (no further bleeding) and biological (hemoglobin level, von Willebrand factor parameters) improvements. Because of the persistence of bleeding risk factors, the treatment was maintained at a prophylactic dose (20 UI/kg three times a week), without recurrence of bleeding events for a period of 9 months.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140190436","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Novel Perspectives on Thrombopoietin Receptor Agonists Applications.","authors":"Christos Stafylidis, Dimitra Vlachopoulou, Stavroula Syriopoulou, Sevastianos Chatzidavid, Nora-Athina Viniou","doi":"10.1055/a-2250-9369","DOIUrl":"https://doi.org/10.1055/a-2250-9369","url":null,"abstract":"<p><p>Second-generation thrombopoietin receptor agonists (TPO-RAs), romiplostim, eltrombopag, and avatrombopag, have been proved to be significant stimulators of megakaryopoiesis and, in the last decade, they have been incorporated in the treatment options against refractory immune thrombocytopenia in children and adults that do not respond to conventional therapy. Additionally, given their beneficial impact on hematopoiesis, they have successfully been applied in cases of non-immune thrombocytopenia, such as aplastic anemia, HCV-related thrombocytopenia, chronic liver disease, and most recently acute radiation syndrome. During the past years, a wide variety of clinical studies have been performed, in regard to the use of TPO-RAs in various thrombocytopenic settings, such as malignant hematology and hematopoietic stem cell transplantation, hereditary thrombocytopenias, and chemotherapy-treated patients with solid organ tumors. Although data indicate that TPO-RAs may be an effective and safe option for managing disease- or treatment-related thrombocytopenia in these patients, further research is needed to determine their efficacy and safety in these settings. Furthermore, recent studies have highlighted novel properties of TPO-RAs that render them as potential treatment candidates for reducing tumor burden or fighting infections. Herein, we discuss the potential novel applications of TPO-RAs and focus on data regarding their efficacy and safety in these contexts.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140159588","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 : 2024-03-11DOI: 10.1055/a-2238-4782
Nikica Sabljic, Mirjana Mitrovic, Nikola Pantic, Jecko Thachil
{"title":"Thrombosis in Acute Promyelocytic Leukemia: The Current Understanding.","authors":"Nikica Sabljic, Mirjana Mitrovic, Nikola Pantic, Jecko Thachil","doi":"10.1055/a-2238-4782","DOIUrl":"https://doi.org/10.1055/a-2238-4782","url":null,"abstract":"<p><p>Despite enormous improvement in the management of patients with acute promyelocytic leukemia (APL), the distinctive coagulopathy observed at presentation in affected patients is often life-threatening. While hemorrhagic manifestations are well known and described in this setting, APL-related thromboses are underappreciated. Data regarding this complication are scarce showing variable incidence. Furthermore, risk factors for thrombosis are inconsistent and unreliable; so, differentiation of increased risk of hemorrhage from an increased thrombotic risk is quite difficult in the absence of adequate predictive scores. Besides, prophylactic use of anticoagulants and recombinant thrombomodulin are a matter of ongoing debate. Also, due to the common feature of thrombocytopenia and other hemorrhagic risks, patients with APL are excluded from trials analyzing anticoagulant prophylaxis in cancers; so, data from prospective trials are lacking. A detailed analysis of thrombotic risks in APL with the development of a reliable risk stratification model is needed to further improve the care of APL patients.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140102903","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 : 2024-03-11DOI: 10.1055/a-2266-8021
Alexander Mair, Gilles Huber, Jan-Dirk Studt, Donat R Spahn, Alexander Kaserer
{"title":"Quantifying Residual Rivaroxaban Plasma Concentration after Antagonization with Andexanet Alfa: A Difficult Task in Routine Clinical Practice.","authors":"Alexander Mair, Gilles Huber, Jan-Dirk Studt, Donat R Spahn, Alexander Kaserer","doi":"10.1055/a-2266-8021","DOIUrl":"https://doi.org/10.1055/a-2266-8021","url":null,"abstract":"<p><p>We describe the case of a 38-year-old man with a history of chronic portal vein thrombosis who presented with abdominal pain after a transjugular intrahepatic portosystemic shunt procedure. Under anticoagulation therapy with rivaroxaban, he experienced active splenic bleeding, leading to hemodynamic instability. Emergency interventions, including andexanet alfa and nanoparticle administration, successfully stopped the bleeding. However, routine tests showed persistently high rivaroxaban levels despite reversal with andexanet alfa. This case report shows that next to standard anti-Xa activity assay, high-performance liquid chromatography is as well unreliable in this regard. In contrast, viscoelastic tests might better serve as indicators of the efficacy of the reversal. The availability of modified anti-Xa tests is urgently needed, to monitor the effects of andexanet alfa reversal.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140102902","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 : 2024-03-06DOI: 10.1055/s-0044-1780535
M Edvardsson, M Oweling, P Järemo
{"title":"Type 2 Diabetes: Platelets and Long-Term Metabolic Control as Estimated from Glycosylated Haemoglobin (HbA1c).","authors":"M Edvardsson, M Oweling, P Järemo","doi":"10.1055/s-0044-1780535","DOIUrl":"https://doi.org/10.1055/s-0044-1780535","url":null,"abstract":"<p><p>In type 2 diabetes, platelets are likely affected by impaired long-term glycaemic control, but such pathophysiological links are poorly understood. This study thus compares platelet reactivity (i.e. agonist-evoked platelet reactions) <i>in vitro</i> with glycosylated haemoglobin (HbA1c), a measure commonly used for monitoring long-term metabolic control of type 2 diabetes. Elders with type 2 diabetes (<i>n</i> = 35) were divided according to HbA1c into groups (HbA1c-low and high) consisting of 17 and 18 subjects, respectively. For estimating mitochondria disintegration, a flow cytometer determined mitochondrial transmembrane potentials after whole blood agonist stimulation. The activating agents used were α-thrombin (10 μM) and collagen (0.15 μg/mL). The same apparatus analysed the fibrinogen receptor activity, lysosomal exocytosis (surface lysosomal-associated membrane protein 1), and platelet procoagulant characteristics (membrane-attached annexin V) after stimulation. In type 2 diabetes, after <i>in vitro</i> agonist stimulation, platelet mitochondria injury was higher in the HbA1c-high group. The fibrinogen receptor, lysosomal secretion, and the creation of procoagulant platelets proved to be uninfluenced by HbA1c.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140051102","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 : 2024-03-01DOI: 10.1055/a-2238-4744
Mirjana Kovac, Milena Todorovic Balint, Marija Milenkovic, Dusica Basaric, Branko Tomic, Bela Balint, Vera Ignjatovic
{"title":"Assessment of Factor VIII Activity and D-Dimer Levels in the Post-COVID Period.","authors":"Mirjana Kovac, Milena Todorovic Balint, Marija Milenkovic, Dusica Basaric, Branko Tomic, Bela Balint, Vera Ignjatovic","doi":"10.1055/a-2238-4744","DOIUrl":"https://doi.org/10.1055/a-2238-4744","url":null,"abstract":"<p><p>Changes in the hemostatic system during COVID infection lead to hypercoagulability. Numerous studies have evaluated hemostatic abnormalities in COVID patients during acute infection, in the period of hospitalization. However, the hemostatic status following hospital discharge has not been sufficiently assessed. Considering the importance of FVIII and D-dimer levels as markers for the assessment of thrombosis, our study aimed to evaluate changes in these markers, as well as the influence of patient's age and clinical presentation of COVID infection on those hemostatic markers in the post-COVID phase. This prospective study (July 2020 to December 2022) included 115 COVID patients, 68 (59%) with asymptomatic/mild and 47 (41%) with moderate/severe clinical presentation. Patient follow-up included laboratory evaluation of FVIII and D-dimer levels at 1, 3, and 6 months following the COVID infection. Three months after the COVID infection, elevated FVIII was recorded in 44% of younger versus 65% of older individuals, <i>p</i> = 0.05, respectively, and 30 versus 57% (<i>p</i> = 0.008) 6 months post-COVID infection. With a focus on clinical presentation, a higher number of patients with moderate/severe COVID had elevated FVIII activity, but a statistically significant difference was observed only for the 6 months (32% mild vs. 53% moderate/severe, <i>p</i> = 0.041) post-infection time point. Following a COVID infection, an increase in FVIII activity suggests a continued hypercoagulable state in the post-COVID period and correlates with elevated D-dimer levels. This increase in FVIII is more pronounced in patients with moderate/severe clinical picture and those patients older than 50 years.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140013679","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 : 2024-03-01DOI: 10.1055/a-2211-6841
Christian Irsara, Andrea Griesmacher, Lorin Loacker, Clemens Feistritzer, Cosima Anna Überbacher, Jean Amiral
{"title":"Anti-Thrombin IgA in a Patient with Multiple Myeloma Leading to In Vitro Interference in Multiple Coagulation Tests and Confounding Diagnosis.","authors":"Christian Irsara, Andrea Griesmacher, Lorin Loacker, Clemens Feistritzer, Cosima Anna Überbacher, Jean Amiral","doi":"10.1055/a-2211-6841","DOIUrl":"10.1055/a-2211-6841","url":null,"abstract":"<p><strong>Background: </strong> We report the case of a 59-year-old multiple myeloma patient in whom an anti-human thrombin IgA antibody led to prolonged in vitro coagulation times, suggesting inhibitors to all intrinsic coagulation factors in the absence of spontaneous bleeding.</p><p><strong>Methods: </strong> Routine and extensive special coagulation tests, in vivo bleeding time, and specific antibody testing were performed.</p><p><strong>Results: </strong> Although the patient did not suffer from spontaneous bleeding and had a normal in vivo bleeding time, the anti-human thrombin IgA autoantibody affected all coagulation assays involving human thrombin in vitro, mimicking inhibitors to intrinsic coagulation factors. As the IgA paraprotein and the IgA antibody virtually disappeared after autologous stem cell transplantation, the coagulation tests also largely normalized.</p><p><strong>Conclusion: </strong> Antibodies to human thrombin may interfere with all coagulation assays involving thrombin, imitating a severe coagulopathy. However, in vivo they do not necessarily lead to strongly increased bleeding tendency. Complex and ambiguous coagulation abnormalities should be evaluated and treated in an interdisciplinary setting, including a highly specialized coagulation laboratory, from the beginning.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140013678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
HamostaseologiePub Date : 2024-02-09DOI: 10.1055/a-2231-5277
Lennart Beckmann, Tobias D Faizy, Fabian Flottmann, Jens Fiehler, Carsten Bokemeyer, Lennart Well, Antonia Beitzen-Heineke, Florian Langer
{"title":"Multisite Thrombosis in a Patient with Paroxysmal Nocturnal Hemoglobinuria.","authors":"Lennart Beckmann, Tobias D Faizy, Fabian Flottmann, Jens Fiehler, Carsten Bokemeyer, Lennart Well, Antonia Beitzen-Heineke, Florian Langer","doi":"10.1055/a-2231-5277","DOIUrl":"https://doi.org/10.1055/a-2231-5277","url":null,"abstract":"<p><strong>Case: </strong>Paroxysmal nocturnal hemoglobinuria (PNH) is an extremely rare bone marrow disorder caused by acquired mutations in the phosphatidylinositol glycan class A gene, which lead to a partial or total loss of the cellular complement regulators CD55 and CD59.1 In addition to complement-mediated hemolysis and cytopenia, venous and arterial thromboses at multiple and/or unusual sites are a common complication and occur in up to 44% of patients in historic PNH cohorts.1 2.</p>","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":3.2,"publicationDate":"2024-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139713467","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}