Seminars in thrombosis and hemostasis
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Semin. Thromb. Hemost. · Jul 2012
ReviewAutoimmune thrombotic microangiopathy: advances in pathogenesis, diagnosis, and management.
Thrombotic microangiopathy, or the syndrome of thrombocytopenia and hemolysis with schistocytes on blood smears, has been a subject of uncertainty and intense controversy. The pathogenesis of thrombotic microangiopathy was unknown and no classification of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome was satisfactory. ⋯ This pathogenesis-based disease classification requires new diagnostic approaches and provides a framework for rational therapeutic designs. This review discusses the new concepts in the pathogenesis, diagnosis, and management of thrombotic microangiopathy, with particular emphasis on the autoimmune causes of ADAMTS-13 deficiency and defective complement regulation.
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Semin. Thromb. Hemost. · Jun 2012
Update on the catastrophic antiphospholipid syndrome and the "CAPS Registry".
Although less than 1% of patients with the antiphospholipid syndrome (APS) develop the catastrophic variant known as catastrophic antiphospholipid syndrome (CAPS), its potentially lethal outcome emphasizes its importance in clinical medicine today. However, the rarity of this variant makes it extraordinarily difficult to study in any systematic way. ⋯ HTM). Currently, this database documents the entire clinical, laboratory, and therapeutic data of more than 350 fully registered patients.
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Coagulation factor I (fibrinogen) plays an essential role in the hemostatic system by bridging activated platelets and being the key substrate for thrombin in establishing a consolidating fibrin network. Fibrinogen is synthesized in the liver and the plasma concentration is 1 to 5-4.0 g/L. During recent 10 years, fibrinogen has been recognized to play an important role in controlling hemorrhage. ⋯ This article provides a description of the biochemistry and mechanisms of fibrinogen as well as the etiology for developing fibrinogen deficiency. Furthermore, it summarizes laboratory and experimental data on the role of fibrinogen in dilutional coagulopathy and addresses laboratory monitoring issues. Finally, it lists retrospective and prospective studies, which have been designed to assess the clinical efficacy and safety of hemostatic intervention with fibrinogen concentrate.
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Semin. Thromb. Hemost. · Apr 2012
ReviewProhemostatic interventions in trauma: resuscitation-associated coagulopathy, acute traumatic coagulopathy, hemostatic resuscitation, and other hemostatic interventions.
Trauma is the most common cause of death in the young and hemorrhage is the most important cause of death in patients with trauma. Recently redefined pathways of inflammation and coagulation, together with hypothermia and acidosis contribute to trauma-associated coagulopathy and aggravation of bleeding. ⋯ Recombinant factor VIIa, fibrinogen and prothrombin complex concentrates, and antifibrinolytic agents have been evaluated in clinical trials. These interventions show promising effects but their efficacy in reducing clinically important outcome parameters need to be confirmed in clinical studies.
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Surgical procedures of the liver, such as partial liver resections and liver transplantation, are major types of abdominal surgery. Liver surgery can be associated with excessive intraoperative blood loss, not only because the liver is a highly vascularized organ, but also because it plays a central role in the hemostatic system. Intraoperative blood loss and transfusion of blood products have been shown to be negatively associated with postoperative outcome after liver surgery. ⋯ In patients with liver cirrhosis, there is increasing evidence that factors such as portal hypertension and the hyperdynamic circulation play a more important role in the bleeding tendency than changes in the coagulation system. Therefore, intravenous fluid restriction rather than prophylactic administration of large volumes of blood products (i.e., FFP) is recommended in patients undergoing major liver surgery. Pharmacological agents such as antifibrinolytic drugs or recombinant factor VIIa may be indicated in selected individual patients, but these agents do not have a routine role in the management of patients undergoing liver surgery.