UNASSIGNED: Thrombophilia combined with pregnancy poses significant risks for adverse pregnancy outcomes. Unfortunately, there are no indicators at high risk for predicting adverse pregnancy outcomes. This study investigates the predictive efficiency of serum immune-inflammatory markers on adverse pregnancy outcomes.
UNASSIGNED: This retrospective cohort study includes 223 pregnant women diagnosed with thrombophilia who delivered at the Fujian Provincial Hospital South Branch from January 2022 to April 2024. Clinical information and pregnancy outcomes were collected. The systemic immune-inflammation index (SII), systemic inflammation response index (SIRI), and lactate dehydrogenase (LDH) were calculated using blood samples. The relationship and predictive accuracy between immune-inflammatory markers and adverse pregnancy outcomes were analyzed.
UNASSIGNED: In this study, 50 (22.4%) patients had adverse pregnancy outcomes. Significant differences were observed in neutrophils counts, monocytes counts, LDH, SII, and SIRI levels between the adverse pregnancy outcome groups (APOs) and the control groups (P<0.05). The area under the receiver operating characteristic (ROC) curve analysis revealed that SII (AUC=0.762), SIRI (AUC=0.764), and LDH (AUC=0.732) had high predictive values for adverse pregnancy outcomes. Notably, the combined model had the highest AUC of 0.805. Multivariate logistic regression identified SII had the highest odd ratio (OR) (OR=8.512; 95% CI(3.068-23.614)), followed by LDH (OR=4.905; 95% CI (1.167-11.101)), SIRI (OR=3.549; 95% CI(0.847-8.669)), and neutrophils count (OR=1.726; 95% CI (0.563-2.938)) as independent risk factors for adverse outcomes.
UNASSIGNED: Elevated levels of immune-inflammatory markers such as SII, SIRI, and LDH level are strong predictors of adverse pregnancy outcomes in thrombophilia-complicated pregnancies. These markers are significantly associated with maternal-neonatal outcomes. Our findings underscore the importance of monitoring immune-inflammatory markers in pregnant women with thrombophilia to improve maternal and neonatal outcomes.

In thrombotic diseases, coagulation, anticoagulation, and fibrinolysis are three key physiological processes that interact to maintain blood in an appropriate state within blood vessels. When these processes become imbalanced, such as excessive coagulation or reduced anticoagulant function, it can lead to the formation of blood clots. Genetic factors play a significant role in the onset of thrombotic diseases and exhibit regional and ethnic variations. The decision of whether to initiate prophylactic anticoagulant therapy is a matter that clinicians must carefully consider, leading to the development of various thrombotic risk assessment scales in clinical practice. Given the considerable heterogeneity in clinical diagnosis and treatment, researchers are exploring the application of artificial intelligence in medicine, including disease prediction, diagnosis, treatment, prevention, and patient management. This paper reviews the research progress on various genetic factors involved in thrombotic diseases, analyzes the advantages and disadvantages of commonly used thrombotic risk assessment scales and the characteristics of ideal scoring scales, and explores the application of artificial intelligence in the medical field, along with its future prospects.

The etiology of childhood arterial ischemic stroke is complex, and identifying the underlying cause is crucial for optimizing treatment and preventing recurrence. Currently, the classification methods for childhood arterial ischemic stroke are largely based on data from international studies, but a unified consensus have not yet been reached. This paper reviews the existing classification methods and their subtype definitions, and points out some doubts and ambiguities. On this basisi, combined with the data collected by Beijing Children\'s Hospital on Chinese children with arterial ischemic stroke, a new classification method (COIST) was proposed according to the etiology and pathogenesis, namely: inflammation (I), abnormal vascular structure (S), thrombophilia (T), heart disease (C), other identifiable causes (O), and uncertain causes; and various subtypes are listed. It is hoped that this new classification method can attract the attention and discussion of domestic colleagues, with the aim of further refinement, in order to help clinicians better understand and quickly identify the etiologies of childhood ischemic stroke.
儿童动脉缺血性脑卒中的病因复杂，识别其病因是优化治疗和预防卒中复发的关键。关于儿童动脉缺血性脑卒中的分型方法，目前多参考国外研究数据，但尚未达成统一。本文梳理了现有的分型方法及其亚型定义，并指出了存在的一些疑问和歧义。在此基础上，结合北京儿童医院收集的中国儿童动脉缺血性卒中的数据，根据病因和发病机制，提出了新的分型方法（COIST分型方法），即：炎症性（I）、血管结构异常（S）、易栓症（T）、心脏疾病（C）、其他可确定病因（O）、原因不能确定；并列出了各亚型疾病。希望这一新的分型方法能引起国内同行的关注和讨论，以期进一步完善，从而方便临床医师更好地了解和快速识别儿童缺血性卒中的病因。.

BACKGROUND: Cholestatic liver diseases induce local and systemic hypercoagulation, with neutrophil extracellular traps (NETs) serving as major drivers. These NETs have been linked to decreased liver function in patients with obstructive jaundice. However, the impact of NETs on liver hypercoagulation in cholestatic liver disease remains unknown.
METHODS: We utilized bile duct ligation to create experimental mice and analyzed NETs formation in the liver. Fibrin deposition, tissue factor expression, and inflammation in the liver were visualized through western blot and immunohistochemical techniques. LSECs were incubated with isolated NETs, and we detected endothelial procoagulant activity using coagulation protein production assays and measuring endothelial permeability. In both in vivo and in vitro settings, DNase I was applied to clarify the effect of NETs on intrahepatic hypercoagulability, hepatotoxicity, LSEC, and macrophage activation or injury.
RESULTS: Bile duct ligation mice exhibited significantly increased levels of NETs in liver tissue, accompanied by neutrophil infiltration, tissue necrosis, fibrin deposition, and thrombophilia compared to sham mice. Notably, NETs resulted in phosphatidylserine and tissue factor exposure on LSEC, enhancing coagulation Factor Xa and thrombin production. The enhanced procoagulant activity could be reversed by degrading NETs with DNase I. Additionally, NETs-induced permeability changes in LSECs, characterized by increased VE-cadherin expression and F-actin retraction, which could be rescued by DNase I. Meanwhile, NET formation is associated with KC activation and the formation of inflammatory factors.
CONCLUSIONS: NETs promote intrahepatic activation of coagulation and inflammation, leading to liver tissue injury. Strategies targeting NET formation may offer a potential therapeutic approach for treating cholestatic liver disease.

OBJECTIVE: The prothrombin (PT) G20210A mutation is one of the most prevalent genetic variations associated with an increased susceptibility to the first episode of venous thromboembolism (VTE). However, it remains uncertain whether this inherited thrombophilic abnormality also poses a risk for recurrent VTE. This meta-analysis aimed to assess the relation of PT G20210A mutation to the risk of recurrent VTE.
METHODS: PubMed and Scopus were systematically searched for pertinent prospective studies. Relative risks (RR) and 95% confidence intervals (CI) were used to test the association. Sixteen studies, with 16,174 participants, were included.
RESULTS: Carriers of the G20210 A mutation were at increased risk of recurrent VTE (RR = 1.60, 95% CI = 1.20-2.14) compared to noncarriers; the increased risk was observed in heterozygotes (GA versus GG) (RR = 1.79, 95% CI = 1.24-2.57), but not in GA/AA mutation.
CONCLUSIONS: This association was found to be significant in the long term (≥5 years of follow-up), but not in the short term (<5 years of follow-up).

BACKGROUND: Inherited antithrombin, protein C, and protein S deficiency increase the risk of venous thromboembolism. The presence of defects can be identified by clinical laboratory assays. In most Chinese clinical laboratories, the screening tests for antithrombin, protein C, and protein S deficiency are their activity assays. Ensuring appropriate pre-analytical storage conditions for activity tests is essential. This study aimed to assess the effects of storage conditions on antithrombin, protein C, and protein S activity in frozen plasma.
METHODS: We collected the remaining plasma of 29 patients. The baseline of antithrombin, protein C, and protein S activity values were tested within 4 h. Then, each sample was sub-packaged into 4 EP tubes, and was stored at -20 °C for 3 days, -20 °C for 7 days, -80 °C for 3 days, and - 80 °C for 7 days, respectively. After thawing, samples were tested by two systems.
RESULTS: The percentage deviation of antithrombin and protein C activity assay was<10% compared with the initial values. Protein S activity showed a significant reduction in frozen plasma, with a deviation > 10%. Some samples, initially within the normal range, were classified as abnormal after freezing storage.
CONCLUSIONS: Our study indicated that antithrombin and protein C remain stable when stored at -20 °C or -80 °C in a week. We argued that Protein S activity is not stable in frozen plasma. The use of frozen-thawed plasma for PS activity assay may result in overdiagnosis of protein S deficiency.

BACKGROUND: The phenomenon of hypercoagulability has not been previously documented in individuals with Morvan\'s syndrome, especially in those associated with contactin-associated protein-like receptor 2 (CASPR2).
METHODS: A previously healthy 32-year-old Chinese male was admitted to the hospital with central and peripheral neurologic symptoms. The patient was tested positive for anti-CASPR2 antibodies, and also presented with an activated coagulation state on admission, characterized by a low activated partial thromboplastin time and a high platelet count. With gradual improvement of clinical symptoms, activated partial thromboplastin time, and platelet count returned to normal. Simultaneously, anti-CASPR2 antibody titers significantly decreased and eventually became undetectable.
METHODS: The patient was diagnosed as Morvan\'s syndrome with positive anti-CASPAR2 antibodies accompanied with hypercoagulable state.
METHODS: Plasmapheresis was administered to improve the symptoms combined with prednisolone acetate therapy.
RESULTS: The patient experienced complete resolution of all symptoms during hospitalization and generally recovery after 2 months of discharge.
CONCLUSIONS: Emphasis should be directed towards hypercoagulability in individuals diagnosed with Morvan\'s syndrome, particularly those presenting with positive anti-CASPR2 antibodies. Anticoagulant therapy may represent a novel therapeutic approach for individuals afflicted with Morvan\'s syndrome and exhibiting positivity for anti-CASPR2 antibodies.

UNASSIGNED: Patients with idiopathic membranous nephropathy (IMN) are more likely to be complicated by venous thromboembolism (VTE). The aim of the study was to investigate the potential association between anti-phospholipase A2 receptor (PLA2R) antibodies and hypercoagulability in patients with IMN.
UNASSIGNED: A total of 168 patients with biopsy-proven IMN and 36 patients with biopsy-proven minimal change disease (MCD) were enrolled in this study. The clinical data, serum anti-PLA2R antibodies and coagulation-related indices of the patients were retrospectively analyzed.
UNASSIGNED: Patients with IMN were categorized into glomerular PLA2R staining-positive (GAg+) IMN group and glomerular PLA2R staining-negative (GAg-) IMN group in the study. Patients with IMN who were GAg + had lower PT, APTT and R time than patients with IMN who were GAg-, while the CI value was higher in patients with IMN who were GAg+. Patients with IMN who were GAg + were divided into the SAb+/GAg + group and the SAb-/GAg + group. Patients with IMN who were SAb+/GAg + had higher Fib and MA values than patients with IMN who were SAb-/GAg+. Correlation analysis showed that serum anti-PLA2R antibodies were positively correlated with fibrinogen, D-dimer, K time, CI value, α-angle, and MA value. Multiple linear regression analysis indicated that anti-PLA2R antibodies were independently correlated with fibrinogen and MA value.
UNASSIGNED: Our study provides a new perspective on the underlying mechanisms of hypercoagulability in patients with IMN. Anti-PLA2R antibodies are associated with hypercoagulability in patients with IMN and may affect coagulation in patients with IMN by affecting platelet aggregation function and fibrinogen counts.

OBJECTIVE: To investigate the risk factors and underlying causes of pregnancy-related cerebral venous thrombosis (PCVT).
METHODS: A retrospective cohort of 16 patients diagnosed with CVT during pregnancy and postpartum (within six weeks after delivery) in a comprehensive hospital in China between 2009 and 2022 were carefully reviewed, focusing on demographic, clinical, and etiological characteristics, especially underlying causes. We matched 16 PCVT patients with 64 pregnant and puerperal women without PCVT to explore risk factors and clinical susceptibility to PCVT.
RESULTS: PCVT occurred commonly during the first trimester (43.75%) and the puerperium (37.5%). The frequency of anemia, thrombocytosis and thrombocytopenia during pregnancy, dehydration, and pre-pregnancy anemia was significantly higher in women with PCVT than in those without PCVT (P < 0.05). Among the 16 patients, five were diagnosed with antiphospholipid syndrome and one was diagnosed with systemic lupus erythematosus. Three patients had distinct protein S deficiency and one had protein C deficiency. Whole Exome Sequencing (WES) was performed for five patients and revealed likely pathogenic mutations associated with CVT, including heterozygous PROC c.1218G > A (p. Met406Ile), heterozygous PROS1 c.301C > T (p. Arg101Cys), composite heterozygous mutation in the F8 gene (c.144-1259C > T; c.6724G > A (p. Val2242Met)) and homozygous MTHFR c.677C > T (p. Ala222Val).
CONCLUSIONS: The occurrence of anemia, thrombocytopenia and thrombocytosis during pregnancy, dehydration and pre-pregnancy anemia suggested a greater susceptibility to PCVT. For confirmed PCVT patients, autoimmune diseases, hereditary thrombophilia, and hematological disorders were common causes. Screening for potential etiologies should be paid more attention, as it has implications for treatment and long-term management.

Venous thromboembolism, which is common in cancer patients and accompanies or even precedes malignant tumors, is known as cancer-related thrombosis and is an important cause of cancer- associated death. At present, the exact etiology of the elevated incidence of venous thrombosis in cancer patients remains elusive. Platelets play a crucial role in blood coagulation, which is intimately linked to the development of arterial thrombosis. Additionally, platelets contribute to tumor progression and facilitate immune evasion by tumors. Tumor cells can interact with the coagulation system through various mechanisms, such as producing hemostatic proteins, activating platelets, and directly adhering to normal cells. The relationship between platelets and malignant tumors is also significant. In this review article, we will explore these connections.