Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis
-
Blood Coagul. Fibrinolysis · Sep 2012
Fibrinogen Melbourne: a novel congenital hypodysfibrinogenemia caused by γ326Cys-Phe in the fibrinogen γ chain, presenting as massive splanchnic venous thrombosis.
Congenital dysfibrinogenemias are characterized by structural abnormalities in fibrinogen, which may lead to abnormal function. Fibrinogen has critical roles in coagulation, platelet aggregation and fibrinolysis; accordingly, abnormal fibrinogen function can result in a clinical phenotype, which varies from asymptomatic (around 55%) to bleeding (25%) and/or thrombosis (20%). We describe a novel γ326Cys→Phe mutation in the fibrinogen γ gene causing hypodysfibrinogenemia associated with life-threatening thrombosis in a family from Melbourne, Australia.
-
Blood Coagul. Fibrinolysis · Sep 2012
ReviewAnticoagulant therapy: basic principles, classic approaches and recent developments.
The standard multipotent anticoagulants (unfractionated and low molecular weight heparins, antagonists of vitamin K) are commonly used for treatment and/or prophylaxis of different thrombotic complications, such as deep vein thrombosis, thrombophilia, pulmonary embolism, myocardial infarction, stroke and so on. Advantages and shortcomings of these anticoagulants are considered. The modern tendencies to use small selective direct inhibitors of thrombin or factor Xa are surveyed. The search of the new targets in the coagulation cascade for development of new promising anticoagulants and improvement in antithrombotic therapy is discussed.
-
Blood Coagul. Fibrinolysis · Sep 2012
ReviewLocal fibrin glue and chitosan-based dressings in haemophilia surgery.
Fibrin glue and chitosan-based dressings (CBDs) could be useful local haemostatic agents for severe haemorrhage in persons with haemophilia undergoing surgical procedures. The aim of the present study was to clarify the role of fibrin glue and CBDs in persons with haemophilia. A review of the most recent literature on the topic was performed. ⋯ Although a correct surgical haemostasis can be usually achieved by the infusion of factor concentrate at the right dose, my recommendation for surgeons is always to have fibrin glue and CBDs by their side. CBDs could be better haemostatic dressings for control of haemorrhage than current standards of care. To clarify the real value of fibrin glue and CBDs in surgery of haemophilia in specific situations new well designed prospective comparative studies should be carried out.
-
Blood Coagul. Fibrinolysis · Sep 2012
Case ReportsRotational thromboelastometry analysis and management of life-threatening haemorrhage in isolated craniofacial injury.
Massive haemorrhage from facial fractures is rare but the associated mortality rate is high. Here, we describe a case in which thromboelastometry [rotational thromboelastometry (ROTEM)]-guided administration of prothrombin complex concentrate and fibrinogen concentrate was effective in correcting coagulopathy in a 68-year-old man with serious craniofacial trauma and massive haemorrhage. The patient, a cyclist who collided with a car, was transferred to the emergency department of our hospital with signs of shock and significant bleeding from multiple fractures and soft tissue injuries to the face. ⋯ ROTEM analysis revealed severe coagulopathy and was indispensable in guiding transfusion: 2 g tranexamic acid, followed by 1000 IU prothrombin complex concentrate, 5 g fibrinogen and 2 U platelet concentrate. Two hours later, ROTEM analysis showed that coagulopathy had been corrected, and haemostasis was confirmed by cessation of bleeding. This report highlights the potential for using ROTEM to guide treatment with fibrinogen and prothrombin complex concentrates in the presence of profuse multifocal bleeding and severe coagulopathy.
-
Blood Coagul. Fibrinolysis · Sep 2012
Variability of GP6 gene in patients with sticky platelet syndrome and deep venous thrombosis and/or pulmonary embolism.
The GP6 gene encodes the GPVI, a crucial platelet membrane glycoprotein, for adequate platelet activation, adhesion and aggregation. The objectives of the present study were to assess the genetic variability of the GP6 gene in patients with platelet hyperaggregability phenotype, known as sticky platelet syndrome (SPS) manifesting as deep vein thrombosis (DVT), and/or pulmonary embolism, and in controls; and to evaluate its role in the pathogenesis of venous thromboembolism (VTE) in SPS. Seventy-seven patients with SPS and 77 healthy blood donors as controls were enrolled. ⋯ In the patients, significant positive relationship between the 1671153-G, 1654419-A, 1613662-G alleles and male sex was observed. GP6 SNPs 1613662-G, 1671153-G and 1654419-A alleles are associated with an increased risk of VTE in SPS. They could contribute to the SPS phenotype.