Hamostaseologie最新文献_第2页

High Prevalence of Acquired Platelet Secretion Defects in Multiple Myeloma. 获得性血小板分泌缺陷在多发性骨髓瘤中的高发性。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub 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":"10.1055/a-2418-5664","url":null,"abstract":"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":"272-282"},"PeriodicalIF":2.4,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13498419/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Gemeinsame Stellungnahme zum GKV-Beitragsstabilisierungsgesetz: Qualität der Versorgung sichern. 《食品安全法案》：确保食品安全。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-08-21 DOI: 10.1055/a-2822-5903

Christoph Sucker, Jürgen Koscielny

引用次数: 0

Die Gesellschaft für Thrombose- und Hämostaseforschung e.V. informiert. 血栓和止血研究协会。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-08-21 DOI: 10.1055/a-2822-6063

引用次数: 0

Standards and Practice in Haemostasis. 止血的标准和实践。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-08-21 DOI: 10.1055/a-2822-5925

Heiko Rühl, Manuela Albisetti, Florian Langer

引用次数: 0

Luft-, Lärm- und Lichtverschmutzung als Risikofaktoren für thromboembolische kardiovaskuläre Komplikationen – eine wissenschaftliche Stellungnahme in Thrombosis and Haemostasis. 空气、噪音和光污染作为血栓栓塞心血管并发症的危险因素：血栓形成和止血的科学意见。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-08-01 Epub Date: 2026-08-21 DOI: 10.1055/a-2902-4868

引用次数: 0

Rivaroxaban in Paediatric Patients Beyond Clinical Trial Settings - A Real-World Retrospective Cohort Study. 利伐沙班在儿科患者中的临床试验设置-一个真实世界的回顾性队列研究。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-07-29 DOI: 10.1055/a-2918-8110

Lukas Röthlisberger, Manuela Albisetti, Alessandra Bosch

{"title":"Rivaroxaban in Paediatric Patients Beyond Clinical Trial Settings - A Real-World Retrospective Cohort Study.","authors":"Lukas Röthlisberger, Manuela Albisetti, Alessandra Bosch","doi":"10.1055/a-2918-8110","DOIUrl":"https://doi.org/10.1055/a-2918-8110","url":null,"abstract":"Abstract: BACKGROUND: Direct oral anticoagulants (DOACs) show increasing popularity in the treatment of paediatric venous thromboembolism (VTE), but real-world evidence outside clinical trials remains scarce.Abstract: OBJECTIVES: This study aimed to evaluate the use, safety and effectiveness of rivaroxaban in a real-world paediatric cohort at a tertiary care hospital.Abstract: METHODS: All children exposed to rivaroxaban between January 2020 and June 2025 at our institution were included into this single-centre retrospective cohort study. In chart reviews, effectiveness was assessed as thrombus recurrence, progression, resolution, or improvement; safety outcomes included clinically relevant bleeding (CRB) and minor bleeding.Abstract: RESULTS: Fifty-seven children were included at a median age of 13.8 years (IQR 7.1-15.6); 49% were female. In the treatment group (n = 37), one thrombosis recurred (3%), with thrombus resolution in 19 (54%) and radiologic improvement in 5 patients (14%). In the prophylaxis group (n = 20), no incident VTE occurred. Overall, CRB was rare (7%), all non-major during rivaroxaban exposure and occurred exclusively in children with identifiable additional bleeding risk factors.Abstract: CONCLUSIONS: Rivaroxaban demonstrated a favourable effectiveness and safety profile in this real-world paediatric cohort, with outcomes comparable to available real-world and trial data. CRB events clustered in settings with additional bleeding risks, underscoring the importance of patient characteristics in guiding therapy. Our findings support rivaroxaban as a feasible anticoagulation option in children.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148622487","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

Subcutaneous Treatment Hope: Non-Factor Care in Children with Hemophilia. 皮下治疗的希望：血友病儿童的非因素护理。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-07-15 DOI: 10.1055/a-2901-3232

Katharina Thom

{"title":"Subcutaneous Treatment Hope: Non-Factor Care in Children with Hemophilia.","authors":"Katharina Thom","doi":"10.1055/a-2901-3232","DOIUrl":"https://doi.org/10.1055/a-2901-3232","url":null,"abstract":"Abstract: Hemophilia A or B is an inherited bleeding disorder due to a deficiency of factor VIII or IX. Depending on the severity, bleeding may already occur after birth and in very young patients, e.g., intracranial hemorrhage or bleeding in muscle, soft tissue, and joints during surgery or after little accidents.Abstract: Despite great improvements in factor therapy concerning half-life and less frequent injections, remaining issues are the intravenous administration and inhibitor development. Additionally, a primary regular factor prophylaxis maybe more effective to prevent joint bleeds and damages in adolescence and adults.Abstract: Non-factor therapies have been a so-called \"game changer\" in hemophilia care, most of all due to the subcutaneous application and improved quality of life. Existing data demonstrate a more stable coagulation activation and effective bleeding prevention, also in patients with inhibitors. These novel therapies work either as factor mimetics, providing similarity to the missing factor, or they have a rebalancing effect by targeting natural coagulation inhibitors, such as tissue factor pathway inhibitor, or protein C. Depending on data and the approval situation, these therapies may allow an easier and earlier start of prophylaxis and potentially prevent early bleeding. However, concerns about these new treatments include, e.g., risk of coagulation imbalances and insufficient monitoring.Abstract: This review will comprise an overview of non-factor treatment, such as FVIII mimetics and re-balancing therapies, including working profile, pediatric data, and approval situation in Europe, Switzerland, and the United States.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-07-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148457671","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

Update: Immune Tolerance Induction Practice in Haemophilia. 更新：血友病免疫耐受诱导实践。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-07-06 DOI: 10.1055/a-2887-8098

Carmen Escuriola Ettingshausen

{"title":"Update: Immune Tolerance Induction Practice in Haemophilia.","authors":"Carmen Escuriola Ettingshausen","doi":"10.1055/a-2887-8098","DOIUrl":"https://doi.org/10.1055/a-2887-8098","url":null,"abstract":"Abstract: The emergence of non-factor therapies has fundamentally changed inhibitor management in haemophilia. Historically, immune tolerance induction (ITI) was considered indispensable for eradicating factor VIII (FVIII) or IX inhibitors and restoring responsiveness to replacement therapy. However, the introduction of rebalancing therapies (TFPI inhibitors, tissue factor pathway inhibitors) and emicizumab, a bispecific antibody mimicking factor VIIIa activity, provides highly effective bleed protection regardless of inhibitor status, challenging the traditional paradigm of universal ITI. Subcutaneous emicizumab and TFPI inhibitors enable safe, convenient prophylaxis without the need for central venous access or intensive FVIII respectively IX exposure, raising the question of whether ITI remains necessary in all patients with haemophilia A or B with inhibitors.Abstract: Current ITI practice increasingly favours combined ITI-emicizumab strategies, which reduce treatment burden and bleeding risk. Interim data from the MOTIVATE study and registry data indicate that combined approaches can achieve inhibitor eradication, yet success rates and optimal protocols remain uncertain. Moreover, the clinical relevance of tolerance is debated: while FVIII treatment is effective and safe to treat major bleeds or to cover major surgery compared to bypassing agents, many patients achieve good bleed protection with emicizumab prophylaxis without inhibitor eradication, but data on long-term joint health are still lacking. In haemophilia B, ITI feasibility is further limited by allergic reactions and nephrotic syndrome, making rebalancing therapies as a promising alternative for long-term care. This review explores whether ITI continues to be justified in the era of non-factor prophylaxis, summarizing evolving strategies, real-world evidence and unresolved questions.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-07-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148399104","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

Too Common to Ignore: Decoding the Complexity of the FGG p.Ala108Gly Variant in Fibrinogen Disorders. 太普遍而不能忽视：解码纤维蛋白原疾病中FGG p.a ala108gly变异的复杂性。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-06-22 DOI: 10.1055/a-2819-9796

Daniel Alexander Jørgensen, Mustafa Vakur Bor

引用次数: 0

Diagnosis and Management of Immune Platelet Transfusion Refractoriness. 免疫血小板输注难治性的诊断与处理。

IF 2.4 4区医学

Hamostaseologie Pub Date : 2026-06-22 DOI: 10.1055/a-2887-8181

Sara Reda, Heiko Rühl

{"title":"Diagnosis and Management of Immune Platelet Transfusion Refractoriness.","authors":"Sara Reda, Heiko Rühl","doi":"10.1055/a-2887-8181","DOIUrl":"https://doi.org/10.1055/a-2887-8181","url":null,"abstract":"Abstract: Platelet transfusion refractoriness (PTR) represents a major challenge in the transfusion support of patients with severe thrombocytopenia, particularly those with hematologic malignancies, intensive chemotherapy, or hematopoietic stem cell transplantation. Although nonimmune mechanisms account for the majority of cases, immune-mediated PTR caused by alloantibodies against platelet antigens, most commonly human leukocyte antigen (HLA) class I molecules, remains clinically important. This review summarizes current knowledge on the definition, mechanisms, diagnostic evaluation, and management of immune PTR. The immunologic basis of platelet destruction in immune PTR involves several mechanisms mediated by anti-HLA antibodies, including Fc receptor-dependent phagocytosis, complement activation, and antibody-induced platelet activation. Diagnostic evaluation integrates clinical assessment with laboratory detection of HLA antibodies, molecular HLA typing, and platelet crossmatching. Clinical management primarily relies on the provision of compatible platelet products, including HLA-matched platelets, antibody-specific antigen-negative platelet units, or crossmatched platelet concentrates. In selected cases, additional testing for antibodies against human platelet antigens (HPAs) may be required. Preventive strategies such as leukoreduction have substantially reduced the incidence of alloimmunization, although immune PTR continues to occur in a subset of transfusion-dependent patients. Emerging strategies aimed at improving transfusion outcomes include approaches to reduce platelet immunogenicity, development of HLA-deficient platelets, and pharmacologic interventions targeting antibody-mediated platelet clearance. A better understanding of the mechanisms underlying immune PTR may help refine diagnostic algorithms and expand therapeutic options for patients requiring long-term platelet transfusion support.","PeriodicalId":55074,"journal":{"name":"Hamostaseologie","volume":" ","pages":""},"PeriodicalIF":2.4,"publicationDate":"2026-06-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148304100","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