Research and Practice in Thrombosis and Haemostasis最新文献

{"title":"Safety and efficacy of direct oral anticoagulants in chronic kidney disease: a meta-analysis","authors":"Daniel Tham ,&nbsp;Lucy Zhao ,&nbsp;Wenhui Yu ,&nbsp;Jayhan Kherani ,&nbsp;Roger Kou ,&nbsp;Allen Li ,&nbsp;Pei Ye Li ,&nbsp;Ali Eshaghpour ,&nbsp;Mark Andrew Crowther","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":"8 7","pages":"Article 102584"},"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":"A cluster of pediatric vaccine-induced immune thrombotic thrombocytopenia–like cases with thrombosis and thrombocytopenia following respiratory infections—case series","authors":"Dimitra Dimopoulou ,&nbsp;Lida Mentesidou ,&nbsp;Athina Dettoraki ,&nbsp;Christina Karastathi ,&nbsp;Maria Berikopoulou ,&nbsp;Panagiota Katsouli ,&nbsp;Ioanna Anastasopoulou ,&nbsp;Iason G. Stamatakis ,&nbsp;Theodora Bachou ,&nbsp;Flora Tzifi ,&nbsp;Aikaterini Michalopoulou ,&nbsp;Anna Messaritaki ,&nbsp;Vana Spoulou ,&nbsp;Helen Pergantou","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":"8 7","pages":"Article 102589"},"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":"Clinical characteristics, treatment, and outcomes of provoked acute cerebral sinovenous thrombosis in patients <21 years old: findings from the Kids-DOTT Multinational Trial","authors":"Gary M. Woods ,&nbsp;Alexandra Miller ,&nbsp;Maua Mosha ,&nbsp;Christoph Male ,&nbsp;Anupam Verma ,&nbsp;Nicole Kucine ,&nbsp;Christine Sabapathy ,&nbsp;Kisha Beg ,&nbsp;Sanjay Ahuja ,&nbsp;Deepti Raybagkar ,&nbsp;Kerry Hege ,&nbsp;Clara Lo ,&nbsp;Rukhmi Bhat ,&nbsp;Thomas Abshire ,&nbsp;Neil A. Goldenberg","doi":"10.1016/j.rpth.2024.102605","DOIUrl":"10.1016/j.rpth.2024.102605","url":null,"abstract":"<div><h3>Background</h3><div>Prospective multicenter data on the treatment and outcomes of children with cerebral sinovenous thrombosis (CSVT) are limited. We aimed to describe the clinical characteristics, treatment strategies, and outcomes of patients with a first-episode of provoked acute CSVT enrolled in the Kids-DOTT trial and compare these features with those of participants with non-CSVT venous thromboembolism (VTE).</div></div><div><h3>Methods</h3><div>This was a subgroup analysis from the Kids-DOTT trial, a multinational randomized clinical trial on duration of anticoagulation for provoked acute VTE in patients younger than 21 years. Patient and thrombus characteristics, treatments, and outcomes of patients diagnosed with CSVT were compared with those of patients with non-CSVT VTE.</div></div><div><h3>Results</h3><div>CSVT was diagnosed in 75 of the 532 (14%), 25 of whom received 6 weeks of anticoagulant treatment and 50 received 3 or more months. When compared with non-CSVT VTE, CSVT was more likely to occur in neonates and young children, associated with infection in general and acute head/neck infection in particular, and less likely to be related to central venous catheter. No patient in either group developed symptomatic recurrent VTE or clinically relevant bleeding, and there was no significant difference in rates of complete thrombus resolution between the 2 treatment durations.</div></div><div><h3>Conclusion</h3><div>CSVT is most common in neonates and young children and those with acute head and neck infections. A 6-week anticoagulation treatment course appears to be safe (no clinically relevant bleeding) and effective (no difference in symptomatic recurrent VTE) for provoked acute pediatric CSVT. Nevertheless, given the nature of a subpopulation analysis, these findings should be interpreted with caution.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102605"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142654130","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":"Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options","authors":"Bülent Zülfikar ,&nbsp;Başak Koç ,&nbsp;Fahri Şahin ,&nbsp;Hatice İlgen Şaşmaz ,&nbsp;Kaan Kavaklı ,&nbsp;Can Balkan ,&nbsp;Ali Bülent Antmen ,&nbsp;Sinan Akbayram ,&nbsp;Birol Güvenç ,&nbsp;Vahap Okan ,&nbsp;Emine Türkkan ,&nbsp;Canan Albayrak ,&nbsp;Davut Albayrak ,&nbsp;Nazan Sarper ,&nbsp;Tülin Tiraje Celkan ,&nbsp;Orhan Ayyıldız ,&nbsp;Salih Aksu ,&nbsp;Türkan Patıroğlu ,&nbsp;Zafer Şalcıoğlu ,&nbsp;Adalet Meral Güneş ,&nbsp;Muhlis Cem Ar","doi":"10.1016/j.rpth.2024.102588","DOIUrl":"10.1016/j.rpth.2024.102588","url":null,"abstract":"<div><h3>Background</h3><div>Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males.</div></div><div><h3>Objectives</h3><div>To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey.</div></div><div><h3>Methods</h3><div>This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018. Analyses were performed by age subgroups (0-17 years, 18-64 years, and ≥65 years), disease severity by factor levels (severe, &lt;1 IU/dL; moderate, 1-5 IU/dL; mild, &gt;5 IU/dL). Additionally, patients were stratified based on the initiation year of follow-up at the relevant study center, creating 2 periods: 1993-2006 (referred to as period A) and 2007-2018 (referred to as period B).</div></div><div><h3>Results</h3><div>Predominantly male (98.6%), the median age at data entry was 22.1 years (<em>n</em> = 429). The majority (49.0%) had moderate HB, followed by severe (30.0%) and mild (15.7%) disease. Of the 377 patients with complete treatment details, 209 (55.4%) were under prophylaxis from their diagnosis onwards, while 79 patients (21.0%) only received on-demand treatment. Additionally, 89 patients (23.6%) initially underwent on-demand treatment and later were switched to prophylaxis. Knees were the primary site of bleeding and the most frequently intervened joints. Most of the major (47.5%) and minor (53.3%) orthopedic procedures were carried out in persons with severe HB, while half of radioactive synovectomy procedures were performed on persons with moderate HB.</div></div><div><h3>Conclusion</h3><div>This paper describes the demographics, clinical characteristics, and treatments patterns of a large cohort of alive persons with HB on a national scale.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102588"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142654132","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":"Danilo Menichelli ,&nbsp;Luca Crisanti ,&nbsp;Tommaso Brogi ,&nbsp;Gregory Y.H. Lip ,&nbsp;Alessio Farcomeni ,&nbsp;Pasquale Pignatelli ,&nbsp;Daniele Pastori","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":"8 7","pages":"Article 102575"},"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}

{"title":"Functional characterization of a nanobody-based glycoprotein VI-specific platelet agonist","authors":"Minka Zivkovic ,&nbsp;Elisabeth Pols - van Veen ,&nbsp;Vossa van der Vegte ,&nbsp;Silvie A.E. Sebastian ,&nbsp;Annick S. de Moor ,&nbsp;Suzanne J.A. Korporaal ,&nbsp;Roger E.G. Schutgens ,&nbsp;Rolf T. Urbanus","doi":"10.1016/j.rpth.2024.102582","DOIUrl":"10.1016/j.rpth.2024.102582","url":null,"abstract":"<div><h3>Background</h3><div>Glycoprotein (GP)VI is a platelet-specific collagen receptor required for platelet activation during hemostasis. Platelet reactivity toward collagen is routinely assessed during diagnostic workup of platelet disorders. GPVI can be activated by inducing receptor clustering with suspensions of fibrillar collagen or synthetic cross-linked collagen-related peptide (CRP-XL). However, these suspensions are poorly standardized or difficult to produce. Nanobodies are small recombinant camelid-derived heavy-chain antibody variable regions. They are highly stable, specific, and ideal candidates for developing a stable GPVI agonist for diagnostic assays.</div></div><div><h3>Objectives</h3><div>Develop a stable nanobody-based GPVI agonist.</div></div><div><h3>Methods</h3><div>Nanobody D2 (NbD2) was produced as dimers and purified. Tetramers were generated via C-terminal fusion of dimers with click chemistry. Nanobody constructs were functionally characterized with light transmission aggregometry (LTA) in platelet-rich plasma and whole blood flow cytometry. Diagnostic performance was assessed in patients with inherited platelet function disorders with LTA and flow cytometry.</div></div><div><h3>Results</h3><div>NbD2 was specific for human platelet GPVI. Dimers did not result in platelet activation in LTA or flow cytometry settings and fully inhibited CRP-XL-induced P-selectin expression and fibrinogen binding in whole blood and attenuated collagen-induced platelet aggregation in platelet-rich plasma. However, NbD2 tetramers caused full platelet aggregation, as well as P-selectin expression and fibrinogen binding. NbD2 tetramers were able to discriminate between inherited platelet function disorder patients and healthy controls based on fibrinogen binding, similar to CRP-XL.</div></div><div><h3>Conclusion</h3><div>Nanobody tetramers to GPVI induce platelet activation and can be used to assess the GPVI pathway in diagnostic assays.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102582"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142535137","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":"Great white sighting: a case of heparin-induced thrombosis with thrombocytosis","authors":"Julia Levy ,&nbsp;Hong De Sa ,&nbsp;Lindsey Loss ,&nbsp;Mandy VanSandt ,&nbsp;Rhusheet Patel ,&nbsp;Merav Sendowski","doi":"10.1016/j.rpth.2024.102587","DOIUrl":"10.1016/j.rpth.2024.102587","url":null,"abstract":"<div><h3>Background</h3><div>Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse response to heparin therapy, characterized by decreased platelet count and increased risk of thrombosis. HIT, without the tell-tale sign of thrombocytopenia, has rarely been described.</div></div><div><h3>Key Clinical Question</h3><div>Can HIT be diagnosed in the presence of thrombocytosis? What clinical clues and diagnostic tools facilitate accurate diagnosis in such cases?</div></div><div><h3>Clinical Approach</h3><div>We report a case of HIT with thrombocytosis in a young male who initially presented after traumatic knee dislocation. HIT was diagnosed clinically through the discovery of a white thrombus during a vascular surgery procedure and corroborated by a positive latex immunoturbidimetric immunoassay (HemosIL HIT-Ab _{(platelet-factor 4(PF4)-heparin)}), a rapid immunoassay.</div></div><div><h3>Conclusion</h3><div>With its high sensitivity, specificity, and rapid results, the latex immunoturbidimetric immunoassay is a valuable diagnostic tool, even among patients with a seemingly low pretest probability. This case underscores the guidance imparted by Dr Andreas Greinacher: “[HIT] must be considered if thrombosis occurs or progresses despite effective heparinization even in the absence of thrombocytopenia.” Access to rapid and effective laboratory testing reduces the probability of diagnostic error.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102587"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142552331","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":"The impact of pulmonary embolism on health outcomes of COVID-19 at 3 months after hospitalization","authors":"Chantal Visser ,&nbsp;Julia C. Berentschot ,&nbsp;Cindy M.M. de Jong ,&nbsp;M. Louisa Antoni ,&nbsp;L. Martine Bek ,&nbsp;Rita J.G. van den Berg-Emons ,&nbsp;Bram van den Borst ,&nbsp;Hugo ten Cate ,&nbsp;Arina J. ten Cate-Hoek ,&nbsp;Dionne C.W. Braeken ,&nbsp;J.J. Miranda Geelhoed ,&nbsp;Majanka H. Heijenbrok-Kal ,&nbsp;Sander M.J. van Kuijk ,&nbsp;Lucia J.M. Kroft ,&nbsp;Jenneke Leentjens ,&nbsp;Anna H.E. Roukens ,&nbsp;Suzanne C. Cannegieter ,&nbsp;Frederikus A. Klok ,&nbsp;Marieke J.H.A. Kruip ,&nbsp;Merel E. Hellemons","doi":"10.1016/j.rpth.2024.102573","DOIUrl":"10.1016/j.rpth.2024.102573","url":null,"abstract":"<div><h3>Background</h3><div>COVID-19 patients frequently experience pulmonary embolism (PE), but its long-term consequences remain uncertain.</div></div><div><h3>Objectives</h3><div>To assess the impact of PE in COVID-19 patients on health outcomes at 3 months after hospitalization.</div></div><div><h3>Methods</h3><div>In this multicenter cross-sectional study, we aggregated data from existing databases to evaluate the impact of PE on health outcomes at 3 months after hospitalization. We assessed 1) questionnaires on health-related quality of life (5-level EuroQol 5-dimensional questionnaire [EQ-5D-5L] questionnaire), anxiety, depression, cognitive failure, and posttraumatic stress disorder; 2) pulmonary function tests (diffusing capacity of the lungs for carbon monoxide [DLCO] and spirometry); and 3) radiological abnormalities. We developed 3 models to assess the association between PE and the EQ-5D-5L index and the percentage of predicted DLCO (DLCO%): a crude model (model 1), adjusted for age, sex, and presence of comorbidities (model 2), and model 2 additionally adjusted for intensive care unit admission (model 3).</div></div><div><h3>Results</h3><div>We included 465 patients who had been hospitalized for COVID-19, of whom 102 (21.9%) had developed a PE during admission. Patients with PE had poorer EQ-5D-5L index values, more impairment in pulmonary functions, and more frequent radiological abnormalities than patients without PE. Symptoms of anxiety, depression, cognitive failure, and posttraumatic stress disorder did not differ between the 2 groups. In model 2, PE was associated with lower EQ-5D-5L index and lower DLCO%. After additionally adjusting for intensive care unit admission, the association between PE and lower EQ-5D-5L index (mean difference = −0.069, [95% CI, −0.12 to −0.017]) remained but not between PE and DLCO%.</div></div><div><h3>Conclusion</h3><div>Our findings suggest that PE in COVID-19 patients is associated with reduced health-related quality of life at 3 months after hospitalization. While PE may be a marker of COVID-19 severity, its presence during hospitalization could indicate potential long-term health issues, which may be considered during follow-up care.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102573"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142419949","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":"Periprocedural hemostatic prophylaxis and outcomes in bleeding disorder of unknown cause","authors":"Callie Berkowitz ,&nbsp;Alice Ma ,&nbsp;Vanessa Miller ,&nbsp;Supreet Goraya ,&nbsp;Kristi Kirkland ,&nbsp;Julie Grabell ,&nbsp;Nigel S. Key ,&nbsp;Paula D. James","doi":"10.1016/j.rpth.2024.102572","DOIUrl":"10.1016/j.rpth.2024.102572","url":null,"abstract":"<div><h3>Background</h3><div>Bleeding disorder of unknown cause (BDUC) is a diagnostic category encompassing patients with a clear bleeding phenotype but without identifiable abnormality on hemostatic testing. The optimal management of hemostasis in BDUC patients prior to invasive procedures and childbirth is uncertain.</div></div><div><h3>Objectives</h3><div>Our objective was to characterize periprocedural hemostatic prophylaxis and bleeding outcomes in patients with BDUC.</div></div><div><h3>Methods</h3><div>We conducted a retrospective cohort study of adult patients with BDUC at 2 academic medical centers. Following diagnosis of BDUC, subsequent surgical procedures and childbirths were analyzed using a combination of registry data and manual chart review.</div></div><div><h3>Results</h3><div>We identified 127 patients with mean age of 39.9 years (SD = 16.6); the majority of patients were female (91.3%). Forty-eight major procedures, 70 minor procedures, and 19 childbirths were analyzed. Antifibrinolytic monotherapy was advised for 57% of major procedures, 59% of minor procedures, and 67% of childbirths. Perioperative platelet transfusion was recommended in 26% of major procedures and 9% of minor procedures in combination with other hemostatic agents. Major or clinically relevant nonmajor bleeding occurred in 4.1% (4/98) of procedures with prophylaxis and 10% (2/20) of procedures without prophylaxis. Postpartum hemorrhage occurred in 26% (5/19) of deliveries.</div></div><div><h3>Conclusion</h3><div>In this multiinstitution experience, we found overall low rates of hemostatic complications in procedures completed with hemostatic prophylaxis, although preventing hemorrhage in childbirth and gynecologic procedures remain unmet needs.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102572"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142535135","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":"Prediction of the chance of successful immune tolerance induction in persons with severe hemophilia A and inhibitors: a clinical prediction model","authors":"Ilja Oomen ,&nbsp;Amal Abdi ,&nbsp;Ricardo M. Camelo ,&nbsp;Fábia M.R.A. Callado ,&nbsp;Luany E.M. Carvalho ,&nbsp;Ilenia L. Calcaterra ,&nbsp;Manuel Carcao ,&nbsp;Giancarlo Castaman ,&nbsp;Jeroen C.J. Eikenboom ,&nbsp;Kathelijn Fischer ,&nbsp;Vivian K.B. Franco ,&nbsp;Martijn W. Heymans ,&nbsp;Frank W.G. Leebeek ,&nbsp;David Lillicrap ,&nbsp;Cláudia S. Lorenzato ,&nbsp;Maria Elisa Mancuso ,&nbsp;Davide Matino ,&nbsp;Matteo N.D. Di Minno ,&nbsp;Alex B. Mohseny ,&nbsp;Johannes Oldenburg ,&nbsp;Samantha C. Gouw","doi":"10.1016/j.rpth.2024.102580","DOIUrl":"10.1016/j.rpth.2024.102580","url":null,"abstract":"<div><h3>Background</h3><div>Inhibitor eradication to restore factor (F)VIII efficacy is the treatment goal for persons with severe hemophilia A (HA) and inhibitors. Immune tolerance induction (ITI) is demanding and successful in about 70% of people. Until now, it has remained difficult to quantify the probability of ITI success or failure, complicating the decision to initiate or not initiate ITI. Estimating the individual chance of ITI success allows clinicians, patients, and their families to support shared decision-making.</div></div><div><h3>Objectives</h3><div>We aimed to identify clinical predictors of ITI success and to develop a clinical prediction model to estimate the chance of successful ITI in persons with severe HA.</div></div><div><h3>Methods</h3><div>This multicenter study included persons with severe HA who received ITI. Clinical data were collected. Successful ITI was defined by a negative inhibitor titer and an adequate response to FVIII concentrates. A multivariable logistic regression model was developed. Model performance and internal validation were performed.</div></div><div><h3>Results</h3><div>Of 206 participants with a median age of 19.8 months (IQR, 12.1-38.8) at ITI start, 148 (71.8%) achieved ITI success. Our clinical prediction model included 4 predictors of ITI success: cumulative number of FVIII exposure days at inhibitor development, peak inhibitor titer, ethnicity, and <em>F8</em> mutation type. The C statistic was 0.801 (95% CI, 0.70-0.87).</div></div><div><h3>Conclusion</h3><div>In our study, including 206 people with severe HA and inhibitors, we developed a clinical prediction model to estimate the chance of successful ITI. After future external validation, this clinical prediction model may be useful for informing clinicians and families.</div></div>","PeriodicalId":20893,"journal":{"name":"Research and Practice in Thrombosis and Haemostasis","volume":"8 7","pages":"Article 102580"},"PeriodicalIF":3.4,"publicationDate":"2024-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142592790","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}