Research and Practice in Thrombosis and Haemostasis最新文献

{"title":"Bleeding and quality of life in people with Glanzmann thrombasthenia—insights from the Glanzmann’s 360 study","authors":"","doi":"10.1016/j.rpth.2024.102586","DOIUrl":"10.1016/j.rpth.2024.102586","url":null,"abstract":"<div><h3>Background</h3><div>Glanzmann thrombasthenia (GT) is a rare platelet function disorder that results in severe bleeding. We assessed clinical symptoms and psychological parameters to identify the unmet needs associated with GT.</div></div><div><h3>Objectives</h3><div>Glanzmann’s 360 is a mixed-methods study designed to give a contemporary snapshot of the impact of living with GT.</div></div><div><h3>Methods</h3><div>The study comprised a self-completion online survey complemented by interviews conducted with affected individuals and carers recruited via social media and hemophilia treatment centers.</div></div><div><h3>Results</h3><div>The survey was completed by 88 people with GT and 29 carers of children/young people with GT aged &lt;16 years. The population ranged in age from &lt;2 years to &gt;70 years; 56% were female. Although 47% had been diagnosed with GT under the age of 2 years, 12% were diagnosed after 20 years of age. For 82%, a bleeding phenotype was apparent by the age of 5 years. Most respondents (88%) had experienced at least one bleed in the past week. Bleeding disproportionally affected women. Bleeds resulted in frequent hospital contact and considerable psychological distress: 26% of the population had scores suggestive of low self-esteem, while 30% met criteria suggestive of symptomatic depression. Exploratory analyses suggest that bleed experiences are associated with impaired health-related quality of life.</div></div><div><h3>Conclusion</h3><div>The Glanzmann’s 360 study reveals the significant physical, psychosocial, and quality-of-life impairments that are likely to be linked to the frequent bleeds experienced by those with GT. Clinicians treating people with GT should promote access to multidisciplinary comprehensive care, including psychosocial support.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142552330","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Clinical features and treatment of 70 children with lupus anticoagulant-hypoprothrombinemia syndrome: a retrospective study from a single center in China","authors":"","doi":"10.1016/j.rpth.2024.102577","DOIUrl":"10.1016/j.rpth.2024.102577","url":null,"abstract":"<div><h3>Background</h3><div>Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare acquired bleeding disorder characterized by the presence of lupus anticoagulant (LA) and acquired hypoprothrombinemia.</div></div><div><h3>Objectives</h3><div>To summarize the experience of diagnosis, clinical features, and treatment of lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS).</div></div><div><h3>Methods</h3><div>A retrospective study of 70 children diagnosed with LAHPS from January 2019 to February 2024 at a single center was conducted.</div></div><div><h3>Results</h3><div>A total of 70 subjects (32 boys and 38 girls), with a mean age of 5.58 years, were included in the study. Among these subjects, 15 had autoimmune diseases (AIDs), 51 had infections, and 4 had unknown causes. Fifty-six of 70 (80%) subjects experienced bleeding with the median bleeding score of 4, 1 of 70 (1.4%) presented with thrombosis, and 13 of 70 (18.6%) were asymptomatic. All patients exhibited prolonged prothrombin time, significantly prolonged activated partial thromboplastin time, decreased factor (F)II activity (FII:C), and positive lupus anticoagulant. There was a weak negative correlation between the severity of bleeding and FII:C level (<em>rs</em> = −0.4283; <em>P</em> &lt; .001). Patients with infection-associated LAHPS were younger than those with AIDs-associated LAHPS (<em>P</em> &lt; .0001). In the study, LAHPS subjects are treated with corticosteroids as the first-line therapy, or in combination with immunosuppressants. Coagulation factor replacement therapy can effectively prevent and control bleeding events. After follow-up, lupus anticoagulant of all patients had turned negative within 12 weeks. And, prothrombin time and FII:C were completely normalized of all patients without recurrence of bleeding and without thrombosis.</div></div><div><h3>Conclusion</h3><div>Children develop LAHPS most commonly after AIDs and infection. Most patients presented with mild to moderate bleeding. The severity of bleeding symptoms was not exactly parallel to the decreased FII:C level.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142445225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of the VTE-PREDICT calculator on clinicians’ decision making in fictional patients with venous thromboembolism: a randomized controlled trial","authors":"","doi":"10.1016/j.rpth.2024.102569","DOIUrl":"10.1016/j.rpth.2024.102569","url":null,"abstract":"<div><h3>Background</h3><div>After 3 months of anticoagulation for venous thromboembolism (VTE), the decision needs to be made whether to stop anticoagulation or extend treatment indefinitely. The VTE-PREDICT calculator can be used to estimate individual risks of VTE recurrence and bleeding to guide this decision.</div></div><div><h3>Objectives</h3><div>To evaluate the impact of predicted individual risks of recurrence and bleeding on clinicians’ decisions on anticoagulation duration and to assess usefulness of the VTE-PREDICT calculator.</div></div><div><h3>Methods</h3><div>A randomized controlled trial and within-subject study was conducted among clinicians treating VTE patients. The clinicians were asked to complete an online survey containing 6 fictional case vignettes. Group A proposed anticoagulant duration for each case without additional information first and subsequently after seeing calculator-predicted risks (within-subject analysis). Group B was directly provided with calculator risks and proposed treatment duration for each case vignette (for comparison with group A results in a randomized controlled trial analysis). Then, group B received questions on usefulness and credibility of the calculator.</div></div><div><h3>Results</h3><div>Forty-five clinicians were assigned to group A and 48 to B. Overall, group A did not propose different anticoagulation durations than group B. However, individual clinicians in group A changed proposed duration in 35% of the cases after seeing the calculator risks. The calculator was considered useful and credible by most clinicians.</div></div><div><h3>Conclusion</h3><div>Overall, use of the VTE-PREDICT calculator did not affect proposed anticoagulation duration. However, individual clinicians frequently changed their proposed duration after using the calculator, especially for patients with high bleeding risk.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142419948","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"How to treat patients with bleeding disorder of unknown cause?","authors":"","doi":"10.1016/j.rpth.2024.102585","DOIUrl":"10.1016/j.rpth.2024.102585","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142552328","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Multiple myeloma: retrospective assessment of routine thromboprophylaxis and utility of thrombotic risk scores","authors":"","doi":"10.1016/j.rpth.2024.102571","DOIUrl":"10.1016/j.rpth.2024.102571","url":null,"abstract":"<div><h3>Background</h3><div>The high risk of venous thromboembolism (VTE) in multiple myeloma (MM) warrants primary thromboprophylaxis for most patients. Myeloma-specific thrombotic risk scores (TRSs), such as IMPEDE-VTE, SAVED, and PRISM, were developed to improve risk assessment and guide antithrombotic strategies. Their performance is variable and has not yet been tested in Latin America.</div></div><div><h3>Objectives</h3><div>We aimed to assess the use of primary thromboprophylaxis, the incidence of VTE and bleeding events, and the effectiveness of TRSs in patients with newly diagnosed MM.</div></div><div><h3>Methods</h3><div>This was a retrospective, single-center study. Cumulative VTE rates and TRS performance were analyzed using survival and receiver operating characteristic curves.</div></div><div><h3>Results</h3><div>The study included 250 newly diagnosed MM patients; the vast majority (98.6%) received aspirin as thromboprophylaxis. VTE occurred in 8% within the initial 6 months, increasing to 14.8% over a median follow-up of 19 months. High rates of major bleeding (4.8%) and clinically relevant nonmajor bleeding (4.4%) events were documented. A minimal proportion (0.8%, 0.5%, and 1.2%) of patients were classified as low risk by IMPEDE-VTE, PRISM, and SAVED scores, respectively. Only IMPEDE-VTE exhibited a trend for distinguishing between intermediate-risk (7.14%) and high-risk (13.2%) groups (<em>P</em> = .09). PRISM and SAVED scores showed limited utility. VTE did not impact survival.</div></div><div><h3>Conclusion</h3><div>Aspirin as primary thromboprophylaxis carries an unacceptable risk of VTE and bleeding in patients at intermediate or high thrombotic risk. The IMPEDE-VTE score performed best, although without reaching statistical significance. We confirm that VTE does not portend poor overall survival in MM.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142552329","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hyperfibrinolysis: a crucial phenotypic abnormality of posttraumatic fibrinolytic dysfunction","authors":"","doi":"10.1016/j.rpth.2024.102568","DOIUrl":"10.1016/j.rpth.2024.102568","url":null,"abstract":"<div><h3>Background</h3><div>Traumatic fibrinolytic dysfunction is often categorized into 3 phenotypes based on the result of thromboelastography (TEG) lysis at 30 minutes (LY30): fibrinolysis shutdown, physiologic fibrinolysis, and hyperfibrinolysis. However, the molecular pathophysiology of fibrinolytic dysfunction and the association with clinical outcomes have not been fully evaluated.</div></div><div><h3>Objectives</h3><div>To assess whether posttraumatic fibrinolysis phenotypes identified by TEG correlate with levels of key fibrinolysis-related serum markers and with risk of mortality and hospital complications.</div></div><div><h3>Methods</h3><div>This is a secondary analysis of the Pragmatic, Randomized Optimal Platelet and Plasma Ratios trial. Patients were stratified according to the degree of fibrinolysis upon arrival using TEG LY30 values: low LY30, &lt;0.8%; normal LY30, 0.81% to 0.9%; and high LY30, ≥3%. Serial values of molecular markers (0-72 hours after admission) and clinical outcomes were compared between fibrinolysis groups.</div></div><div><h3>Results</h3><div>A total of 547 patients were included (low LY30, 320; normal LY30, 108; high LY30, 119). The high LY30 group had higher tissue plasminogen activator and plasmin-antiplasmin values upon hospital arrival than the low LY30 or normal LY30 groups (<em>P</em> &lt; .001, respectively). There was no significant difference in levels of tissue plasminogen activator, plasmin-antiplasmin, and plasminogen activator inhibitor 1 between the low LY30 and normal LY30 groups. The high LY30 group was associated with an increased risk of 24-hour and 30-day mortality, while there was no significant difference in mortality between the low LY30 and normal LY30 groups.</div></div><div><h3>Conclusion</h3><div>Our results suggest that hyperfibrinolysis is the most common form of traumatic fibrinolytic dysfunction and is associated with worse outcome.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142419946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparable hemostatic capacity of blood taken from the portal vein compared with systemic blood in patients with cirrhosis","authors":"","doi":"10.1016/j.rpth.2024.102583","DOIUrl":"10.1016/j.rpth.2024.102583","url":null,"abstract":"","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142571819","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A cluster of pediatric vaccine-induced immune thrombotic thrombocytopenia–like cases with thrombosis and thrombocytopenia following respiratory infections—case series","authors":"","doi":"10.1016/j.rpth.2024.102589","DOIUrl":"10.1016/j.rpth.2024.102589","url":null,"abstract":"<div><h3>Background</h3><div>Adenoviral vector COVID-19 vaccine-induced immune thrombotic thrombocytopenia (VITT) is a heparin-independent platelet-activating disorder. An increasing number of VITT-like disorders without previous vaccination are being identified.</div></div><div><h3>Key Clinical Question</h3><div>To explore the association of the pediatric cluster of postinfectious thrombosis and thrombocytopenia with VITT-like disorders.</div></div><div><h3>Clinical Approach</h3><div>Three children with severe thrombocytopenia, coagulopathy, elevated D-dimer, and thrombotic events (cerebral venous sinus thrombosis) were reported. Two had positive nasopharyngeal samples for adenovirus, and 1 had group A streptococcus infection. They all had a COVID-19 history and low-risk antiphospholipid syndrome. Heterozygosity for factor V Leiden was found in 2 children. In 2 patients for whom anti–platelet factor 4 (PF4) serology was performed, positive results were found by PF4/polyanion lateral-flow immunoassay but negative results by PF4/polyanion chemiluminescence immunoassay. All patients were treated with enoxaparin or fondaparinux and intravenous immunoglobulin, while 3 received platelets transfusion and steroids.</div></div><div><h3>Conclusion</h3><div>This cluster of pediatric cases with thrombosis and thrombocytopenia may indicate a postinfectious (most notably, postadenovirus) VITT-like disorder.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142586293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and efficacy of direct oral anticoagulants in chronic kidney disease: a meta-analysis","authors":"","doi":"10.1016/j.rpth.2024.102584","DOIUrl":"10.1016/j.rpth.2024.102584","url":null,"abstract":"<div><h3>Background</h3><div>Direct oral anticoagulants (DOACs) have emerged as the first-line therapy for venous thromboembolism and stroke prophylaxis in atrial fibrillation. As DOACs are partially excreted renally, their safety in patients with chronic kidney disease (CKD) is unclear.</div></div><div><h3>Objectives</h3><div>To synthesize primary evidence on the safety profile of DOACs in patients with CKD.</div></div><div><h3>Methods</h3><div>We searched MEDLINE and Embase from inception to June 2023 for randomized and nonrandomized cohort studies comparing DOACs with vitamin K antagonists (VKAs) in CKD patients. Screening and data collection were conducted in duplicate. The primary safety outcome was major bleeding, defined by International Society on Thrombosis and Haemostasis criteria, stratified by CKD severity. Meta-analysis was done using the Mantel–Haenszel random-effects model, presented as odds ratios (ORs) with corresponding 95% CIs.</div></div><div><h3>Results</h3><div>Of the 2355 articles captured in the literature search, 25 nonrandomized studies (<em>n</em> = 6832) and 6 randomized studies (<em>n</em> = 66,898) were included. DOACs reduced major bleeding compared with VKAs in all subgroups (stage 4: OR, 0.73; 95% CI, 0.58, 0.93; stage 5/renal replacement therapy: OR, 0.70; 95% CI, 0.50, 0.98; stage unspecified: OR, 0.72; 95% CI, 0.63, 0.83). Apixaban and rivaroxaban both reduced major bleeding in stage 5/renal replacement therapy patients (apixaban: OR, 0.66; 95% CI, 0.52, 0.85; rivaroxaban: OR, 0.58; 95% CI, 0.35, 0.94).</div></div><div><h3>Conclusion</h3><div>In this meta-analysis, DOACs reduced major bleeding compared with VKAs in stage 4, stage 5/renal replacement therapy, and CKD stage unspecified patients. Future analysis should evaluate the impact of specific DOACs and dosage on safety and efficacy in this population.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142592789","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incident thrombocytopenia and bleeding risk in elderly patients with atrial fibrillation on direct oral anticoagulants: insights from the ATHEROsclerosis in Atrial Fibrillation study","authors":"","doi":"10.1016/j.rpth.2024.102575","DOIUrl":"10.1016/j.rpth.2024.102575","url":null,"abstract":"<div><h3>Background</h3><div>The bleeding risk of patients with atrial fibrillation (AF) changes over time. Most studies thus far evaluated only the baseline bleeding risk with discordant results. The impact of incident thrombocytopenia during direct oral anticoagulant (DOAC) therapy and its relation to bleeding has not been previously investigated.</div></div><div><h3>Objectives</h3><div>To investigate the incidence rate of thrombocytopenia and major bleeding (MB) risk in AF patients on DOACs.</div></div><div><h3>Methods</h3><div>Prospective ongoing ATHEROsclerosis in Atrial Fibrillation study including patients with nonvalvular AF on DOACs. Incident thrombocytopenia was defined as a platelet count &lt;150 × 10⁹/L. MB events were recorded at each follow-up visit. Gray estimator for competing risk data was used. Estimates are expressed in terms of subdistributional hazard ratios (sHR) and relative 95% CI for MB.</div></div><div><h3>Results</h3><div>We enrolled 957 AF patients treated with DOACs (mean age, 77.3 ± 9.0 years; 49.1% women). During a follow-up (median time to censoring 1330 days; 95% CI, 1246-1443), 139 patients developed thrombocytopenia (3.08 per 100 person-years; 95% CI, 2.27-3.89) with no difference between direct thrombin and factor Xa inhibitors. Overall, 179 bleedings occurred, of which 80 were major (3.17 per 100 person-years; 95% CI, 2.34-3.99). Patients sustaining bleedings were more frequently affected by arterial hypertension, heart failure, anemia and had higher CHA₂DS₂-VASc and HAS-BLED scores. On multivariable Cox analysis, independent risk factors for MB were incident thrombocytopenia (sHR, 12.77; 95% CI, 8.880-18.360; <em>P</em> &lt; .001), and age (sHR, 1.030 per year; 95% CI, 1.010-1.040; <em>P</em> = .002).</div></div><div><h3>Conclusion</h3><div>Patients developing thrombocytopenia have an increased risk of MB. Dynamic evaluation of platelet count during follow-up may provide better prognostic value than baseline assessment only.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":null,"pages":null},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142419950","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}