Hamostaseologie最新文献

Pathophysiology and Management of Cerebral Venous Thrombosis.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-04-08 DOI: 10.1055/a-2518-9103

Mariana B Morais, Diana Aguiar de Sousa

引用次数: 0

Platelet Reduction in Rats Exposed to Chronic Hypoxia Is Associated with Interaction of Glycoprotein Ib Alpha von Willebrand Factor.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-03-28 DOI: 10.1055/a-2462-6667

Zhuoya Wang, Drolma Gomchok, Yi Ye, Yi Wen, Tana Wuren

{"title":"Platelet Reduction in Rats Exposed to Chronic Hypoxia Is Associated with Interaction of Glycoprotein Ib Alpha von Willebrand Factor.","authors":"Zhuoya Wang, Drolma Gomchok, Yi Ye, Yi Wen, Tana Wuren","doi":"10.1055/a-2462-6667","DOIUrl":"https://doi.org/10.1055/a-2462-6667","url":null,"abstract":"Chronic high-altitude hypoxia is associated with reduced platelet count, but it is unclear whether the decrease in platelet count is due to impaired production or increased clearance. This study examines how hypoxia affects platelet production and apoptosis and elucidates the impact of glycoprotein Ibα-von Willebrand factor interaction on platelets in rats using a hypobaric hypoxia chamber. The results showed that the number of megakaryocytes increased under hypoxia; however, the levels of differentiation and polyploidy decreased, while those of apoptosis increased. Platelet production did not reduce according to the reticulated platelet percentage, while platelet apoptosis enhanced; these results suggest that increased platelet clearance was the main reason behind platelet reduction. Our previous microarray results indicated that glycoprotein Ibα (GPIbα) expression increased under hypoxia, which was a protein involved in platelet clearance; therefore, we examined the interaction of platelet GPIbα with the von Willebrand factor (vWF) both in vivo and in vitro to explore the effect of this process on platelets and whether it is related to platelet apoptosis. Under hypoxia, the stronger interaction between GPIbα and vWF promoted platelet apoptosis; inhibiting this interaction reduced platelet apoptosis and increased platelet counts. Platelet reduction is associated with apoptosis induced by the interaction between GPIbα and vWF.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143744470","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}

引用次数: 0

Correlation between Platelet-to-Lymphocyte Ratio, Neutrophil-to-Lymphocyte Ratio and Burden of Thrombus with Disease Severity in Patients with Pulmonary Thromboembolism.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-03-28 DOI: 10.1055/a-2506-6705

Ayshan Mammadova, Kübra Taşkaraca, Günel Jeyranova, Aysel Orujlu, Merve Tatlılıoğlu, Serra Duygulu, Zeynep Yalçınkaya, Seriyye Allahverdiyeva, Onur Gündoğdu, Atiye Cenay Karabörk Kılıç, Sevcihan Kesen Özbek, Gonca Erbaş, I Kıvılcım Oğuzülgen

{"title":"Correlation between Platelet-to-Lymphocyte Ratio, Neutrophil-to-Lymphocyte Ratio and Burden of Thrombus with Disease Severity in Patients with Pulmonary Thromboembolism.","authors":"Ayshan Mammadova, Kübra Taşkaraca, Günel Jeyranova, Aysel Orujlu, Merve Tatlılıoğlu, Serra Duygulu, Zeynep Yalçınkaya, Seriyye Allahverdiyeva, Onur Gündoğdu, Atiye Cenay Karabörk Kılıç, Sevcihan Kesen Özbek, Gonca Erbaş, I Kıvılcım Oğuzülgen","doi":"10.1055/a-2506-6705","DOIUrl":"https://doi.org/10.1055/a-2506-6705","url":null,"abstract":"High neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) are markers of subclinical inflammation and have been associated with prognosis and mortality in many diseases. In this study, we evaluated the comparative value of NLR and PLR in identifying high mortality risk in patients hospitalized with acute pulmonary thromboembolism (PTE), and their relationship with the anatomical burden of thrombus.Patients who were followed up due to PTE were evaluated retrospectively. NLR and PLR were calculated from complete blood counts. The thrombus burden was assessed by the Qanadli score; based on the patients' archival computed tomography angiography images. Mortality prediction was based on an algorithm using the Pulmonary Embolism Severity Index, echocardiographic findings, and troponin levels.Three hundred-two PTE patients were included in the study. Median NLR, PLR, and Qanadli score values were higher in nonsurvivors, with NLR (8.4 [2.2-18.9]) vs. (3.1 [0.4-13.1]), PLR (317 [87.6-525.3]) vs. (124.4 [30-476.3]), and Qanadli scores (21 [3-26]) vs. (9 [1-28]), respectively (p < 0.001). We showed that setting a threshold value of >4.45 for NLR and >151.59 for PLR significantly predicts the high mortality-risk group. In the receiver operating characteristic analysis, when the threshold value for the Qanadli score distinguishing between low-risk and high-risk disease was set at 15.5, the sensitivity was calculated as 98.8% and the specificity was 94.9% (p = 0.001).This study showed that NLR, PLR, and Qanadli scores can provide essential contributions to the clinician's determination of the anatomical burden of thrombus and disease severity in PTE patients.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143744466","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}

引用次数: 0

Erratum to: Congenital Fibrinogen Deficiencies: Not So Rare.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-03-13 DOI: 10.1055/s-0045-1806916

Alexander Couzens, Marguerite Neerman-Arbez

引用次数: 0

Congenital Fibrinogen Deficiencies: Not So Rare.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-03-12 DOI: 10.1055/a-2511-3314

Alexander Couzens, Marguerite Neerman-Arbez

{"title":"Congenital Fibrinogen Deficiencies: Not So Rare.","authors":"Alexander Couzens, Marguerite Neerman-Arbez","doi":"10.1055/a-2511-3314","DOIUrl":"10.1055/a-2511-3314","url":null,"abstract":"Congenital fibrinogen deficiencies (CFDs), traditionally considered rare monogenic disorders, are now recognized as more prevalent and genetically complex than previously thought. Indeed, the symptoms manifested in CFD patients, such as bleeding and thrombosis, are likely to result from variation in several genes rather than solely driven by variants in one of the three fibrinogen genes, FGB, FGA, and FGG. This review highlights recent advances in understanding the genetic causes of CFD and their variability, facilitated by the growing use and availability of next-generation sequencing data. Using gnomAD v4.1.0. data, which includes more than 800,000 individuals, we provide updated global prevalence estimates for CFDs based on frequencies of predicted deleterious variants in FGB, FGA, and FGG. Recessively inherited fibrinogen deficiencies (homozygous genotypes) could be present in around 29 individuals per million, while dominantly inherited deficiencies (heterozygous genotypes) may be present in up to 15,000 per million. These increased estimates can be attributed to the inclusion of broader, more diverse genetic datasets in the new version of gnomAD, thus capturing a greater range of rare variants and homozygous cases.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143617855","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}

引用次数: 0

Changes in Hemophilia Treatment in the Eastern Part of Germany between 2015 and 2021-Data from the Kompetenznetz Hämorrhagische Diathese Ost (KHDO).

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-02-18 DOI: 10.1055/a-2499-2912

Christian Pfrepper, Annika Siecke, Robert Klamroth, Caspar Kühnöl, Karim Kentouche, Susanne Holzhauer, Lars Fischer, Volker Aumann, Karolin Trautmann-Grill, Ute Scholz, Ines Halm-Heinrich, Beate Krammer-Steiner, Kristina Schilling, Ralf Knöfler, Nikola Hagedorn

{"title":"Changes in Hemophilia Treatment in the Eastern Part of Germany between 2015 and 2021-Data from the Kompetenznetz Hämorrhagische Diathese Ost (KHDO).","authors":"Christian Pfrepper, Annika Siecke, Robert Klamroth, Caspar Kühnöl, Karim Kentouche, Susanne Holzhauer, Lars Fischer, Volker Aumann, Karolin Trautmann-Grill, Ute Scholz, Ines Halm-Heinrich, Beate Krammer-Steiner, Kristina Schilling, Ralf Knöfler, Nikola Hagedorn","doi":"10.1055/a-2499-2912","DOIUrl":"https://doi.org/10.1055/a-2499-2912","url":null,"abstract":"Introduction: Treatment options for patients with hemophilia (PWH) have changed substantially in the last years. This study aimed to compare hemophilia treatment in the eastern part of Germany in 2021 with data from 2015.Methods: Substitution diaries and patient records of PWH from 2021 were collected in 13 hemophilia centers from the \"Kompetenznetz Hämorrhagische Diathese Ost\" (KHDO) and compared with 2015.Results: A total of 130 children and 357 adults, 411 hemophilia A (HA) and 76 hemophilia B (HB), were included in 2021, and 359 were already analyzed in 2015. In 2021, 97.8% of children and 95.7% of adults with severe hemophilia had prophylaxis compared with 98.8 and 80.2% in 2015. Plasma-derived concentrates were used in 25.6%, recombinant in 30.2%, extended half-life (EHL) factor concentrates in 24.4%, and emicizumab in 19.8% of the children with severe HA (sHA). In adults with sHA, plasma-derived, recombinant concentrates, EHL, and emicizumab were used in 21.0, 33.2, 31.2, and 14.2%, respectively. All children and 93.3% of the adults with severe HB (sHB) were on EHL. Median annual factor consumption per body weight increased in adults with sHA, remained stable in children with sHA and adults with sHB, and decreased in children with sHB between 2015 and 2021. Annualized bleeding rate (ABR) decreased in children with sHB and sHA.Conclusion: The use of EHL and emicizumab has changed hemophilia treatment. About 50% of the sHA patients switched to EHL or emicizumab and almost all sHB patients to EHL. More adults with sHA received prophylaxis and ABR decreased in children.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143451070","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}

引用次数: 0

High Prevalence of Acquired Platelet Secretion Defects in Multiple Myeloma.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-02-11 DOI: 10.1055/a-2418-5664

Frauke Swieringa, Yaqiu Sang, Jasper A Remijn, Rob Fijnheer, Suzanne J A Korporaal, Rolf T Urbanus, Dana Huskens, Joke Konings, Li Li, Bas de Laat, Mark Roest

{"title":"High Prevalence of Acquired Platelet Secretion Defects in Multiple Myeloma.","authors":"Frauke Swieringa, Yaqiu Sang, Jasper A Remijn, Rob Fijnheer, Suzanne J A Korporaal, Rolf T Urbanus, Dana Huskens, Joke Konings, Li Li, Bas de Laat, Mark Roest","doi":"10.1055/a-2418-5664","DOIUrl":"https://doi.org/10.1055/a-2418-5664","url":null,"abstract":"Thrombocytopenia at admission predicts mortality in multiple myeloma (MM) and might link to disease progression. Although thrombocytopenia is known to be associated with MM, a possible thrombopathy is clinically less known. We conducted a case-control study comparing platelet responses of MM patients to controls via flow cytometry, integrin αIIbβ3 activation and P-selectin exposure, and a bioluminescent assay, ATP release. No difference was found at baseline, but upon platelet stimulation, MM patients had decreased αIIbβ3 activation, partly impaired P-selectin exposure, and reduced δ-granule (ATP) secretion. Aspirin treatment in patients did not account for these diminished platelet responses. In total, 29% of patients had thrombocytopenia, while 60% had decreased αIIbβ3 activation and 67% had reduced platelet secretion capacity. Importantly, as secretion capacity was corrected for platelet count, granule release per platelet was reduced in patients versus controls. Of 6 patients with thrombocytopenia 4 displayed a thrombopathy, while for 15 patients with normal count, 64% had reduced αIIbβ3 activation and 73% had reduced platelet secretion capacity. Of all patients, 10% had thrombocytopenia combined with reduced αIIbβ3 activation plus low secretion capacity (one patient showed no qualitative or quantitative platelet defect). Our data suggest that beyond the known thrombocytopenia, MM patients also have reduced platelet function, which could reflect impaired platelet vitality. Combined measurement of platelet count and function, especially secretion capacity, gives a more comprehensive view of platelet phenotype than count alone. Large prospective follow-up studies are needed to confirm the importance of the acquired platelet secretion defect on the prognosis of MM patients.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-02-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143400778","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}

引用次数: 0

Thrombosis at Unusual Sites: Focus on Myeloproliferative Neoplasms and Paroxysmal Nocturnal Hemoglobinuria.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-02-03 DOI: 10.1055/a-2482-3997

Steffen Koschmieder, Jens Panse

{"title":"Thrombosis at Unusual Sites: Focus on Myeloproliferative Neoplasms and Paroxysmal Nocturnal Hemoglobinuria.","authors":"Steffen Koschmieder, Jens Panse","doi":"10.1055/a-2482-3997","DOIUrl":"https://doi.org/10.1055/a-2482-3997","url":null,"abstract":"Patients with thrombosis at an unusual site will need to be explored for rare causes of thrombosis. Two of these rare causes include myeloproliferative neoplasms (MPNs) and paroxysmal nocturnal hemoglobinuria (PNH). It is important not to overlook these causes, since they require specific management, in addition to antithrombotic treatment (anticoagulants, antiplatelet agents). Unusual sites of venous thrombosis include upper extremity veins, splanchnic veins, cerebral veins, and retinal veins, and unusual sites of arterial thrombosis include renal, adrenal, splenic and mesenteric arteries, and intracardiac and aortal locations. Suspicion for MPN and PNH should be raised if there are concomitant abnormalities, such as elevated or decreased blood cell counts or splenomegaly. Diagnosis of MPN and PNH should include JAK2V617F mutational screening as well as flow cytometric assessment of GPI-anchored proteins in the peripheral blood, respectively. Specific treatments for MPN may include phlebotomy or cytoreductive drugs such as hydroxyurea, anagrelide, pegylated interferon-alpha, or Janus kinase inhibitors. Drugs used for PNH treatment include terminal complement inhibitors, such as eculizumab and ravulizumab, as well as proximally acting inhibitors such as pegcetacoplan or iptacopan. Patients with MPN and PNH are at high risk for thrombosis during their entire lifetime and should thus be followed by specialists experienced in the care of these diseases.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.7,"publicationDate":"2025-02-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143124200","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}

引用次数: 0

GTH 2025 ARTE: The Art in Science - Curiosity and Creativity.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-02-01 Epub Date: 2025-02-19 DOI: 10.1055/a-2343-6618

Manuela Albisetti, Lorenzo Alberio

引用次数: 0

Combining Diagnostics and Research in an Academic Laboratory.

IF 2.7 4区医学

Hamostaseologie Pub Date : 2025-02-01 Epub Date: 2025-02-19 DOI: 10.1055/a-2491-3668

Grit Waitz, Stefan Handtke, Carlo Zaninetti, Linda Schönborn, Thomas Thiele

引用次数: 0