Dataset Information

Monocyte activation and acquired autoimmune protein S deficiency promote disseminated intravascular coagulation in a patient with primary antiphospholipid syndrome.

ABSTRACT: Autoimmune protein S (PS) deficiency is a highly thrombotic, potentially life-threatening disorder. Its pathophysiological relevance in the context of primary antiphospholipid syndrome (APS) is unclear. Here, we report the case of a 76-year-old woman, who presented with a painful reticular skin erythema caused by microvascular thromboses. Disseminated intravascular coagulation (DIC) with consumptive coagulopathy was controlled only by continuous anticoagulation. While significantly elevated IgM antibodies to cardiolipin and β₂-glycoprotein-I were consistent with primary APS, a function-blocking PS autoantibody of the IgG isotype was detected. Robust microvesicle (MV)-associated tissue factor (TF) procoagulant activity (PCA) was isolated from patient plasma. Moreover, patient IgG, but not IgM, induced expression of TF PCA and release of TF-bearing MVs by peripheral blood mononuclear cells from healthy donors. In primary APS, induction of monocyte TF in combination with an acquired PS inhibitor may provoke a deleterious imbalance of procoagulant and anticoagulant pathways with evolution of thrombotic DIC.

SUBMITTER: Beckmann L

PROVIDER: S-EPMC8265818 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature

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Monocyte activation and acquired autoimmune protein S deficiency promote disseminated intravascular coagulation in a patient with primary antiphospholipid syndrome.

Beckmann Lennart L Voigtlaender Minna M Holstein Katharina K Lennartz Maximilian M Schneider Stefan W SW Haddad Munif M Renné Thomas T Bokemeyer Carsten C Rolling Christina C CC Langer Florian F

Research and practice in thrombosis and haemostasis 20210628 5

Autoimmune protein S (PS) deficiency is a highly thrombotic, potentially life-threatening disorder. Its pathophysiological relevance in the context of primary antiphospholipid syndrome (APS) is unclear. Here, we report the case of a 76-year-old woman, who presented with a painful reticular skin erythema caused by microvascular thromboses. Disseminated intravascular coagulation (DIC) with consumptive coagulopathy was controlled only by continuous anticoagulation. While significantly elevated IgM ...[more]

PMID: 34263105

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Project description:IntroductionSince disseminated intravascular coagulation (DIC) was first described, it has been considered a serious disease of the coagulation system and a major challenge to clinicians. Currently, several important knowledge gaps remain. The DANish Disseminated Intravascular Coagulation (DANDIC) Cohort Study will aim to answer questions regarding the incidence and mortality of patients with DIC including time trends. The study will also identify prognostic factors that may guide personalised prevention and treatment. Furthermore, the study will describe treatment patterns and the safety and effectiveness of various treatment modalities.Methods and analysisWe will establish the DANDIC Cohort using data collected in daily clinical practice from the Central Denmark Region, which covers approximately 1.3 million residents. The study period will encompass 1 January 2011 through 1 July 2021. Potential DIC cases will be identified from the hospital laboratory database, based on coagulation biomarkers, and diagnoses will be adjudicated by medical experts. The dataset will be enriched with detailed clinical data from electronic medical charts on aetiologies, bleeding, microthrombus formation, organ failure, thrombosis, treatments and comorbidities. The dataset will also take advantage of in-hospital data with longitudinal information on laboratory records, transfusions, microbiology and treatments. It will be possible to merge this dataset with other unique Danish health registries with more than 10 years of virtually complete follow-up. The project will use state-of-the-art epidemiological and biostatistical methods.Ethics and disseminationThe project has been approved by the Danish Patient Safety Authority (31-1521-452), the Central Denmark Region (1-45-70-83-21), the Danish Data Protection Agency (1-16-02-258-21) and all the hospital chairs. Register-based studies require no ethical approval in Denmark. The results will be disseminated in international peer-reviewed journals.

Project description:Backgroundsepsis is a life-threatening organ dysfunction caused by an excessive host immunological response to infection. The incidence of sepsis is increasing every year, and sepsis is the primary cause of mortality in intensive care units (ICUs). DIC is a coagulopathy syndrome that causes microvascular and macrovascular thrombosis and increases the risk of bleeding due to consumptive coagulopathy. The pathophysiology of DIC in sepsis is complex, and further research is required to investigate the involved mechanisms and risk factors.Methodthis study is a prognostic analysis of a retrospective cohort. Samples were patients diagnosed with sepsis and admitted to Cipto Mangunkusumo National General Hospital from January 2016 to October 2022. Research subjects were followed until occurrence of DIC during sepsis or recovery from sepsis. The research subjects were selected from medical records using a consecutive total sampling approach. The inclusion criteria were patients aged ≥18 years old and diagnosed with sepsis according to qSOFA criteria with a score of 2. The exclusion criterion was an incomplete medical record. Bivariate and multivariate logistic regression analyses were performed to determine which independent variables contributed to the incidence of DIC and obtain the odds ratios (ORs). p < 0.05 was considered to indicate a statistically significant difference.Resultsa total of 248 patients were included after considering the inclusion and exclusion criteria. Of these, 50 (20.2%) septic patients developed DIC. In the multivariate analysis, albumin ≤2.5 g/dL (OR: 2.363; 95% CI: 1.201-4.649), respiratory infection (OR: 2.414; 95% CI: 1.046-5.571), and antibiotic treatment ≥1 h (OR: 2.181; 95% CI: 1.014-4.689) were associated with DIC development. On the basis of the ROC curve, the area under the curve (AUC) was determined to be 0.705 with 95% CI = (0.631-0.778).Conclusionin our study, the prevalence of DIC in septic patients was 20.2%. Low albumin, respiratory infection, and antibiotic treatment ≥1 h were found to be risk factors for development of DIC in septic patients.

Dataset Information

Monocyte activation and acquired autoimmune protein S deficiency promote disseminated intravascular coagulation in a patient with primary antiphospholipid syndrome.

Publications

Monocyte activation and acquired autoimmune protein S deficiency promote disseminated intravascular coagulation in a patient with primary antiphospholipid syndrome.

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