A multicenter clinical evaluation of the Clot Signature Analyzer (original) (raw)
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Haemophilia : the official journal of the World Federation of Hemophilia, 2015
Our aim was to generate, optimize and validate a self-administered bleeding assessment tool (self-BAT) for von Willebrand disease (VWD). In Phase 1, medical terminology in the expert-administered International Society on Thrombosis and Haemostasis (ISTH)-BAT was converted into a Grade 4 reading level to produce the first version of the Self-BAT which was then optimized to ensure agreement with the ISTH-BAT. In Phase 2, the normal range of bleeding scores (BSs) was determined and test-retest reliability analysed. In Phase 3, the optimized Self-BAT was tested as a screening tool for first time referrals to the Haematology clinic. Bleeding score from the final optimized version of the Self-BAT showed an excellent intra-class correlation coefficient (ICC) of 0.87 with ISTH-BAT BS in Phase 1. In Phase 2, the normal range of BSs for the optimized Self-BAT was determined to be 0 to +5 for females and 0 to +3 for males and excellent test-retest reliability was shown (ICC = 0.95). In Phase 3...
International Journal of Clinical Practice
Bleeding disorders are a major group of hematological disorders, which are highly prevalent in the world. Excessive bleeding can result in serious consequences including hypoperfusion and cardiac arrest. The body has its selfmechanism to control excessive bleeding which is termed hemostasis. Hemostasis is achieved in two major steps, the formation of the primary and secondary hemostatic plugs. Endothelium, platelets, and coagulation factors are three components involved in hemostasis. Endothelium and platelets have a major role in forming the primary hemostatic plug. Consequently, the first step in investigating a bleeding disorder is platelet count. Despite normal platelet count, abnormality in the primary hemostatic plug may arise due to functional defects of the platelets including adhesion, activation, and aggregation. Von Willebrand disease (VWD) is an endothelial defect and the most prevalent inherited defect in coagulation. Abnormalities in the secondary hemostatic plug are l...
Journal of Thrombosis and Haemostasis, 2020
Platelet-type von Willebrand Disease (PT-VWD) is a rare autosomal dominant platelet bleeding disorder, with fifty-five patients reported worldwide so far, probably frequently misdiagnosed. Currently there are no clear guidelines for the diagnosis and management of PT-VWD and this may contribute to misdiagnosis and thus to inappropriate treatment of these patients. This report provides expert opinion-based consensus recommendations for the standardized diagnostic and management approach to PT-VWD. Tests essential in the diagnostic workup are platelet count and size, ristocetin induced platelet agglutination (RIPA) with mixing studies and sequencing of platelet GP1BA gene. Platelet transfusions and VWF-rich concentrates are the most effective treatments. This consensus may help to avoid misdiagnosis and guide appropriate management of patients with this disease.
Thrombin-generating capacity in patients with von Willebrand's disease
Haematologica, 2007
von Willebrand's disease (VWD) is the most common hereditary bleeding disorder. Its severity can be classified on the basis of von Willebrand factor (VWF) and factor VIII (FVIII) plasma levels and according to the clinical relevance of bleeding episodes. However, patients with very low VWF activity may exhibit a mild bleeding tendency. The basis for this heterogeneous clinical expression of the deficit is still poorly understood. We investigated the relationship between thrombin generation and levels of factor VIII, VWF and clinical bleeding tendency.
2021
Introduction: VonWillebrand disease (VWD) is considered the most common autosomal inherited bleeding disorder. Laboratory testing for diagnosis or exclusion of VWD is based on a complex of different diagnostic assays. In the diagnostic workup of patients with suspected VWD, the von Willebrand factor (VWF) multimer assay is one of the most important indicators for VWF quality. This study aims to assess the VWF multimers profile in patients with bleeding tendency and increase knowledge and awareness of VWD laboratory diagnosis in Estonia. Methods: This retrospective study investigated the laboratory results of 131 individuals who were selected from the laboratory information system based on the request of VWF tests profile and 31 healthy volunteers for comparison. Results: Control group, non-VWD patients and patients suspected with VWD type 2N or mild haemophilia A demonstrated normal VWF multimer (VWF:MM) pattern. Patients with low VWF and suspected with VWD type 1 also showed normal...
International Journal of Contemporary Medical Research [IJCMR]
Introduction: Acquired bleeding disorders are a major cause of mortality, both in the developed and developing countries. An acute haemorrhage should be managed immediately with blood products, factor concentrates or anti-fibrinolytics. Investigations to detect coagulopathies typically include baseline screening tests like prothrombin time, activated partial thromboplastin time, platelet count and fibrinogen level. These tests have a long turn around time which frequently lead to a blinded approach towards blood product support leading to under or over transfusion. In contrast, rotational thromboelastometry (ROTEM) which assesses haemostasis from the start of clot formation to fibrinolysis gives earliest results within ten minutes. This study was done to establish a correlation between ROTEM parameters and standard coagulation profile in the context of acquired bleeding disorders. Material and Methods: A total of 138 subjects-70 patients who presented with acquired bleeding disorders and 68 subjects diagnosed to be normal on the basis of a complete coagulation work up were included as the cases and controls respectively. All samples were subjected to standard coagulation profile and ROTEM analysis which included Clotting Time, Clot Formation Time, Alpha Angle, Maximum Clot Firmness and Maximum Lysis. Results: The Maximum Clot Firmness had a very good co relation with serum fibrinogen levels (k value-0.807; p<0.000; Sensitivity-88%; Specificity-92%), and good correlation with platelet count (k value-0.793; p< 0.000; Sensitivity-86%, Specificity-92%), whereas Clot Formation Time showed moderate correlation with aPTT. Clotting time had a poor correlation with prothrombin time and activated partial thromboplastin time. Conclusion: The achievement of haemostasis is a crucial factor for determining patient outcomes in acquired bleeding disorders. The gold standard test to diagnose coagulopathy is the standard coagulation profile. Rotational thromboelastometry correlates well with standard coagulation parameters. This test which is performed on whole blood showed interpretable results within 10 minutes, whereas standard coagulation profile required an average of 45-75 minutes. In view of the good correlation to the standard coagulation profile, it appears that Rotational Thromboelastometry results can be safely used to implement early transfusion therapy for haemorrhage.