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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Obstetric hemorrhage is a major cause of maternal morbidity and mortality. Pregnancy is associated with substantial hemostatic changes, resulting in a relatively hypercoagulable state. Acquired coagulopathy can, however, develop rapidly in severe obstetric hemorrhage. ⋯ Therefore, until efficacy and safety are demonstrated in obstetric hemorrhage, clinicians should be cautious about wholesale adoption of high FFP:RBC ratio products. Applications of transfusion protocols, dedicated to massive obstetric hemorrhage and multidisciplinarily developed, currently remain the best available option. Similarly, while procoagulant agents appear promising in treatment of obstetric hemorrhage, caution is nonetheless warranted as long as clear evidence in the context of obstetric hemorrhage is lacking.
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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.