- Patients with multiple myeloma and monoclonal gammopathy of undetermined significance have variably increased thrombin generation and different sensitivity to the anticoagulant effect of activated protein C. [Journal Article]Thromb Res. 2023 Jan 23; 223:44-52.TR
- CONCLUSIONS: Hypercoagulability was observed in MM and even in MGUS cases with very low monoclonal protein concentration. In MM patients, APC had no effect on TG, but it attenuated TG in MGUS patients.
- Publisher Full Text (DOI)
- Prolonged Bleeding After Dental Extraction Due to Decreased Serum Level of Von Willebrand Factor Caused by Untreated Profound Hypothyroidism. [Journal Article]J Pediatr Hematol Oncol. 2023 Jan 10 [Online ahead of print]JP
- CONCLUSIONS: Delayed diagnosis of hypothyroidism may lead to atypical presentations such as bleeding diathesis. Profound hypothyroidism should be considered in the differential diagnosis of acquired von Willebrand disease to avoid undue treatment.
- Publisher Full Text (DOI)
- Protein S Deficiency with Recurrent Deep Vein Thrombosis and Post Thrombotic Syndrome: A Case Report. [Journal Article]JNMA J Nepal Med Assoc. 2022 Oct 01; 60(254):892-894.JJ
- Protein S is a vitamin K-dependent protein that acts as a break in secondary hemostasis by inactivating activated factor V and activated factor VIII. We report a case of a 40 years old male who had the first episode of deep vein thrombosis of the left lower limb 10 years back, which despite treatment, reoccurred 3 months later in the bilateral lower limb. Thrombophilic screening showed severe pro…
- Publisher Full Text (DOI)
- SARS-CoV-2 infection predicts larger infarct volume in patients with acute ischemic stroke. [Journal Article]
- CONCLUSIONS: SARS-CoV-2 infection seems to play a major role in endothelium activation and infarct volume extension during AIS.
- PMC Free PDF
- Immunothrombosis biomarkers as potential predictive factors of acute respiratory distress syndrome in moderate-to-critical COVID-19: a single-center, retrospective cohort study. [Journal Article]
- CONCLUSIONS: TF and vWF levels potentially predicted ARDS development within 7 days of admission for COVID-19 after adjusting for traditional risk factors. TF correlated with ventilation impairment in COVID-19 ARDS but further prospective studies are needed.
- PMC Free PDF
- HEMOSTASIS DISORDERS IN CORONARY ARTERY DISEASE: A PROSPECTIVE COMPARATIVE STUDY OF 130 PATIENTS. [Journal Article]Georgian Med News. 2022 NovGM
- The laboratory detection of factors that participate in coagulation mechanisms in patients with coronary heart disease may lead to important findings regarding the contribution of endothelial function to atherosclerotic lesions of coronary arteries. The main purpose of this study was to investigate the role of high-sensitivity C-reactive protein (hs-CRP), von Willebrand factor (vWF) activity, thr…
- Treatment with recombinant ADAMTS13, alleviates hypoxia/reoxygenation-induced pathologies in a mouse model of human sickle cell disease. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Collectively, our data provide evidence that relative ADAMTS13 insufficiency in SCD mice is corrected by pharmacologic treatment with rADAMTS13 and provides an effective disease-modifying approach in a human SCD mouse model.
- Publisher Full Text (DOI)
- Beyond the guidelines: how we approach challenging scenarios in the diagnosis and management of von Willebrand disease. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- Although von Willebrand disease (VWD) is the most common inherited bleeding disorder, its diagnosis and management are often challenging. Clinical practice guidelines, developed through systematic review of the medical literature and considering the best available evidence, provide guidance for common clinical scenarios. However, in the clinical setting, patients often present with characteristic…
- Publisher Full Text (DOI)
- Therapeutic potential of fetal liver cells transplantation in hemophilia A mice. [Journal Article]Haematologica. 2023 Jan 26 [Online ahead of print]H
- Hemophilia A (HA) cell therapy approaches in pediatric individuals require suitable factor (F)VIII-producing cells for stable engraftment. Liver sinusoidal endothelial cells (LSEC) and hematopoietic stem cells (HSC) have been demonstrated to be suitable for the treatment of adult HA-mice. However, after transplantation in busulfan (BU)-conditioned newborn mice, adult LSEC/HSC cannot efficiently e…
- Publisher Full Text (DOI)
- The impact of decreased expression of SVEP1 on abnormal neovascularization and poor prognosis in patients with intrahepatic cholangiocarcinoma. [Journal Article]Front Genet. 2022; 13:1127753.FG
- Introduction: Intrahepatic cholangiocarcinoma (ICC) is one of the most highly heterogeneous malignant solid tumors; it is generally insensitive to clinical treatment and has a poor prognosis. Evidence suggests that abnormal neovascularization in the tumor microenvironment is an important cause of treatment resistance as well as recurrence and metastasis, but the key regulatory molecules are still…
- PMC Free PDF
- Acquired Hemophilia A In Adults: A Multicenter Study from Turkey. [Journal Article]Indian J Hematol Blood Transfus. 2023 Jan; 39(1):107-115.IJ
- Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of A…
- Publisher Full Text (DOI)
- Assessment of incidence of cerebral vascular diseases and prediction of stroke risk in chronic obstructive pulmonary disease patients using multimodal biomarkers. [Journal Article]Clin Respir J. 2023 Jan 25 [Online ahead of print]CR
- CONCLUSIONS: COPD patients had an increased risk for stroke that could be assessed on stroke risk screening instruments, DTI, T2 star, duplex sonography, and laboratory investigation and could be correlated with the severity of the disease.
- Publisher Full Text (DOI)
- Little discrepancy between one-stage and chromogenic factor VIII (FVIII)/IX assays in a large international cohort of persons with nonsevere hemophilia A and B. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Little assay discrepancy was observed despite the presence of mutations previously associated with discrepancy, suggesting that the presence and magnitude of assay discrepancy are largely determined by laboratory variables.
- Publisher Full Text (DOI)
- The mechanistic and structural role of von Willebrand factor in endotoxemia-enhanced deep vein thrombosis in mice. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: We observe a key role for VWF in the pathogenesis of inflammation-associated venous thrombosis while providing a more comprehensive insight into the molecular interactions that constitute the architecture of venous thrombi.
- Publisher Full Text (DOI)
- Acute obstetric coagulopathy during postpartum hemorrhage is caused by hyperfibrinolysis and dysfibrinogenemia: an observational cohort study. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Clinically significant hemostatic impairment is uncommon during PPH, but a subgroup of women have a distinct and severe coagulopathy characterized by hyperfibrinolysis, low fibrinogen, and dysfibrinogenemia associated with poor fetal outcomes.
- Publisher Full Text (DOI)
- Different inhibitor incidence for individual factor VIII concentrates in 1076 previously untreated patients with severe hemophilia A: data from the PedNet cohort. [Letter]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- Publisher Full Text (DOI)
- Evaluation of the procoagulant state in chronic immune thrombocytopenia before and after eltrombopag treatment-a prospective cohort study. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Patients with ITP in this study were in a hypercoagulable state, with preactivated platelets, increased thrombin generation potential, and increased levels of factor VIII and von Willebrand factor. Eltrombopag treatment further increased plasma thrombin generation potential but no other hemostatic parameters.
- Publisher Full Text (DOI)
- Impact of first-line use of caplacizumab on treatment outcomes in immune thrombotic thrombocytopenic purpura. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Caplacizumab should be used in first line and until ADAMTS13-remission, lowers iTTP-related mortality and refractoriness, and decreases the number of daily plasma exchange and hospital stay. This trial is registered at www.
- Publisher Full Text (DOI)
- The effect of a next generation factor VIII mimetic bispecific antibody (Mim8) on assays of factor VIII activity and thrombin generation. [Journal Article]J Thromb Haemost. 2023 Jan 02 [Online ahead of print]JT
- CONCLUSIONS: APTT-based assays are sensitive to Mim8 and should not be performed in the presence of the drug. Chromogenic assays containing human proteins or hybrid human/bovine proteins demonstrated variable sensitivity to Mim8. Bovine only chromogenic assays were largely insensitive to the presence of Mim8. Thrombin generation normalized at increased Mim8 concentrations. Modified one-stage and chromogenic assays could be used to quantify the Mim8 concentration in plasma.
- Publisher Full Text (DOI)
- Pre-existing anti-factor VIII immunity alters therapeutic platelet-targeted factor VIII engraftment following busulfan conditioning through cytotoxic CD8 T cells. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: Pre-existng immunity can alter the engraftment of 2bF8Tg-Sca-1+ cells through the cytotoxic CD8 T-cell-mediated pathway. Sufficient eradication of FVIII-primed CD8 T cells is critical for the success of platelet gene therapy in hemophilia A with inhibitors.
- Publisher Full Text (DOI)
- A prospective, multicenter, open-label phase III study of emicizumab prophylaxis in patients with acquired hemophilia A. [Journal Article]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- CONCLUSIONS: These results suggest that emicizumab prophylaxis with the tested dosing regimen and completion criteria may have a favorable benefit-risk profile in PwAHA.
- Publisher Full Text (DOI)
- Communication from the Scientific Standardization Committees of the International Society on Thrombosis and Haemostasias on vascular endothelium-related biomarkers in disseminated intravascular coagulation. [Practice Guideline]J Thromb Haemost. 2022 Dec 22 [Online ahead of print]JT
- Disseminated intravascular coagulation (DIC) is not a disease criterion but a pathomechanistic process that accompanies various underlying diseases. According to the International Society on Thrombosis and Haemostasias definition, endothelial injury is an essential component in addition to systemic coagulation activation. Despite this definition, current diagnostic criteria for DIC do not include…
- Publisher Full Text (DOI)
- The relative risk of ischemic cerebrovascular accident in patients with Von Willebrand disease. [Journal Article]J Investig Med. 2023 Jan 25 [Online ahead of print]JI
- Cerebrovascular accident (CVA) is one of the leading causes of death in the United States. Von Willebrand factor plays an important role in platelet activation and adhesion. It remains unclear whether Von Willebrand disease (vWD) is associated with a decreased risk of developing CVA. The study aimed to compare the relative risk (RR) of CVA in patients with and without vWD. We queried the National…
- Publisher Full Text (DOI)
- Analysis of thrombogenicity under flow reveals new insights into the prothrombotic state of patients with post-COVID syndrome. [Journal Article]
- CONCLUSIONS: Together, these data present a dynamic assay to investigate the prothrombotic state in PCS, which may help unravel the mechanisms involved and/or establish new therapeutic strategies for this condition.
- PMC Free PDF
- Targeting the von Willebrand Factor-ADAMTS-13 axis in sickle cell disease. [Journal Article]J Thromb Haemost. 2023 Jan; 21(1):2-6.JT
- Publisher Full Text (DOI)
- Challenges in the management of women with type 2B von Willebrand disease during pregnancy and the postpartum period: evidence from literature and data from an international registry and physicians' survey-communication from the Scientific and Standardization Committees of the International Society on Thrombosis and Haemostasis. [Journal Article]J Thromb Haemost. 2023 Jan; 21(1):154-163.JT
- CONCLUSIONS: We conclude that an international consensus and guidance are critically required for better care and improved outcomes in this patient cohort.
- Publisher Full Text (DOI)
- Cardioprotective Actions of a Glucagon-like Peptide-1 Receptor Agonist on Hearts Donated After Circulatory Death. [Journal Article]J Am Heart Assoc. 2023 Jan 25 [Online ahead of print]JA
- Background Heart transplantation with a donation after circulatory death (DCD) heart is complicated by substantial organ ischemia and ischemia-reperfusion injury. Exenatide, a glucagon-like peptide-1 receptor agonist, manifests protection against cardiac ischemia-reperfusion injury in other settings. Here we evaluate the effects of exenatide on DCD hearts in juvenile pigs. Methods and Results DCD…
- Publisher Full Text (DOI)
- COVID-19-associated myocardial injury: A case report. [Case Reports]ESC Heart Fail. 2023 Jan 24 [Online ahead of print]EH
- Coronavirus disease 2019 (COVID-19) is often accompanied by pneumonia and can be fatal. We report a case of COVID-19-associated myocardial injury mimicking fulminant myocarditis. Endomyocardial biopsy revealed numerous von Willebrand factor-rich microthrombi with small myocardial necrotic areas, complement deposits in small vessels/microthrombi, and macrophage-predominant interstitial infiltratio…
- Publisher Full Text (DOI)
- Gene therapy for hemophilia: looking beyond factor expression. [Review]Exp Biol Med (Maywood). 2023 Jan 23 [Online ahead of print]EB
- Hemophilia A (factor VIII [FVIII] deficiency) and hemophilia B (factor IX [FIX] deficiency) are the X-linked recessive bleeding disorders that clinically manifest with recurrent bleeding, predominantly into muscles and joints. In its severe presentation, when factor activity is less than 1% of normal, hemophilia presents with spontaneous musculoskeletal bleeds and may progress to debilitating chr…
- Publisher Full Text (DOI)
- In Vitro Treatment of Extracorporeal Membrane Oxygenation Coagulopathy with Recombinant von Willebrand Factor or Lyophilized Platelets. [Journal Article]J Cardiothorac Vasc Anesth. 2022 Dec 30 [Online ahead of print]JC
- CONCLUSIONS: Recombinant vWF and lyophilized platelets may help to restore primary hemostasis in ECMO patients. Future studies should further evaluate the safety and efficacy of these potential therapeutics in ECMO patients.
- Publisher Full Text (DOI)