Thrombosis Journal最新文献

Background: Antiphospholipid syndrome (APS) is a prothrombotic autoimmune disorder in which perioperative factors-particularly cardiopulmonary bypass-may amplify hypercoagulability. Evidence and consensus guidance for APS management around cardiovascular surgery remain limited. A 37-year-old man with a large, highly mobile thrombus in the ascending aorta underwent surgical thrombectomy under cardiopulmonary bypass. Preoperative testing showed only leukocytosis, slightly shortened activated partial thromboplastin time (APTT) and mild D-dimer elevation; all other findings were unremarkable. Ten days postoperatively, removal of a nontunneled right internal jugular central venous catheter (CVC) was unexpectedly impeded by catheter-related thrombosis (CRT) despite prophylactic anticoagulation. Noninvasive maneuvers failed to free the catheter, necessitating open surgical extraction. Subsequent evaluation revealed widespread venous thromboses and confirmed APS, likely underlying both the arterial event and the rapidly developing catheter-associated thrombosis. Aggressive multimodal therapy-including methylprednisolone, enteric-coated aspirin, warfarin, rituximab, unfractionated heparin, and fibrinogenase-achieved clinical stabilization and prevented further events.

Conclusion: This case highlights the challenges in timely diagnosing APS and the management complexity of rapid, postoperative thrombus formation. Clinicians should maintain a high index of suspicion for early postoperative hypercoagulability-even when routine coagulation screens are unrevealing-escalate promptly when CVC removal meets resistance, and consider APS-tailored antithrombotic strategies. The possibility that fibrin sheaths and catheter-associated thrombosis can evolve quickly, even after short indwelling times, warrants vigilance and early multidisciplinary intervention. It also raises concern that, in this context, standard direct oral anticoagulants (DOACs) may be less effective.

Antithrombin (AT) III is a key physiological anticoagulant, and its hereditary deficiency represents one of the most severe forms of inherited thrombophilia. However, as a rare disorder, AT III deficiency is frequently underdiagnosed due to the limitations of current clinical algorithms. In this study, we describe two cases of hereditary AT III deficiency accompanied by multiple venous thromboembolic events: a 39-year-old male with extensive lower limb deep venous thrombosis (DVT) involving the iliac, femoral, and popliteal veins, and a 21-year-old female with intermediate-high risk pulmonary embolism (PE). Laboratory evaluation revealed reduced AT III activity levels of 51.9% and 52.7%, respectively. Quantitative ELISA analysis further confirmed a corresponding reduction in AT antigen levels. Both patients showed suboptimal responses to initial low-molecular-weight heparin treatment but responded favorably to oral rivaroxaban. Genetic testing identified two nonsense mutations in the SERPINC1 gene: NM_000488.4:c.906dupT (p.Glu303Ter), a previously unreported variant, and NM_000488.4:c.481 C > T (p.Arg161Ter), reported here for the first time in an Asian individual. Family analyses confirmed that the variants were inherited from the proband's parents, who had a history of venous thromboembolism (VTE). These findings underscore the importance of assessing AT activity in patients with unexplained thrombotic events, particularly at a young age, and support the use of genetic testing to guide personalized anticoagulation strategies in AT III deficiency.

Background: Current therapeutic interventions for Sickle Cell Disease (SCD) have improved patient survival; however, the clinical determinants of elevated autoantibody prevalence and related complications in patients with SCD remain insufficiently elucidated.

Objective: This study aimed to examine the frequency of lupus anticoagulant (LA), anticardiolipin antibody (aCL), and anti-beta2 glycoprotein I antibodies (anti- β2GPI) in patients with SCD at steady state phase to evaluate their possible correlations with hematological factors and clinical complications.

Methods: This cross-sectional study involving 92 SCD patients in a steady state phase was conducted. Blood specimens were obtained for complete blood count (CBC), in conjunction with the identification of IgG for aCL and anti-β2GPI utilizing enzyme-linked immunosorbent assay (ELISA) and the assessment of LA through the Diluted Russell Viper Venom Time (dRVVT).

Results: The prevalence of LA, aCL, and anti-β2GPI (IgG) in SCD patients at steady state was 29.4%, 16.3%, and 5.4%, respectively. A correlation was found between LA and chronic leg ulcers, history of thrombosis, decreased platelet counts (PLT), and increased mean platelet volume (MPV) in SCD patients. Furthermore, aCL (IgG) exhibited a significant association with chronic leg ulcers, history of thrombosis, elevated Hemoglobin (Hb) levels, increased red blood cell count (RBC), and higher red cell distribution width (RDW) in SCD patients at steady state phase.

Conclusion: This research elucidates the incidence of LA and aCL in SCD patients during steady state, linking these autoantibodies to hematological parameters and clinical complications; thus, prospective assessments of aPL in SCD patients are essential.

Background: Coagulation dysfunction significantly impacts sepsis prognosis. Standardized methods for evaluating anticoagulant efficacy and safety in this population remain lacking. This study aimed to assess the efficacy and safety of anticoagulant therapy in sepsis patients.

Methods: We systematically searched PubMed, Embase, and Cochrane Library for randomized controlled trials (RCTs) comparing anticoagulants against placebo/no treatment in sepsis patients. The reduction in 28/30-day all-cause mortality or the regression of disseminated intravascular coagulation (DIC) was regarded as efficacy endpoints, while bleeding complications constituted the most prevalent adverse events.

Results: Eighteen RCTs involving 8053 patients were included. Anticoagulant therapy demonstrated an 8% mortality risk reduction versus placebo (relative risk [RR] 0.92, 95% CI 0.86-0.98; P = 0.02). In six studies of baseline DIC patients, anticoagulants showed non-significant mortality reduction (RR 0.87, 95% CI 0.62-1.22; P = 0.42) but significantly enhanced DIC regression (RR 1.62, 95% CI 1.32-2.00; P < 0.00001). Anticoagulants increased bleeding risk (RR 1.32, 95% CI 1.16-1.49; P < 0.0001).

Conclusion: Anticoagulant therapy confers survival benefit in the overall sepsis population despite increased bleeding risk. While improving DIC regression in sepsis-associated DIC, mortality reduction in this subgroup lacked statistical significance. Further research should clarify anticoagulants' role in DIC-specific sepsis management.