Categoría: EHA

Descarga: Treatment of von Willebrand Disease Archivo: eha/vonwill/STH 2016 treatment of VWD.pdf Congenital von Willebrand disease (VWD) and acquired von Willebrand syndrome (AVWS) reflect conditions caused by von Willebrand factor (VWF) deficiency and/or defects.VWDisthemostcommoninheritedbleedingdisorderandAVWSarisesfroma variety of causes. Since VWF stabilizes and protects factor VIII (FVIII) in the circulation, this is also reduced in many patients…

Descarga: The function of ultra-large von Willebrand factor multimers in high shear flow controlled by ADAMTS13 Archivo: eha/vonwill/haemo_2015-The function of ultra-large VWF multimers in .pdf The paradigm that platelet aggregation, which contributes to bleeding arrest and also to thrombovascular disorders, initiates after signaling-induced platelet activation has been refuted in past recent years. Platelets can form…

Descarga: What drives „fibrinolysis“? Archivo: eha/Fibrinolosis/haemo_2015-what drives fibrinolysis.pdf The timely removal of blood clots and fibrin deposits is essential in the regulation of haemostasis. This is achieved by the fibrinolytic system, an enzymatic process that regulates the activation of plasminogen into its proteolytic form, plasmin. This is a self-regulated event as the very presence of…

Descarga: What drives „fibrinolysis“? Archivo: eha/Fibrinolosis/haemo_2015-what drives fibrinolysis.pdf The timely removal of blood clots and fibrin deposits is essential in the regulation of haemostasis. This is achieved by the fibrinolytic system, an enzymatic process that regulates the activation of plasminogen into its proteolytic form, plasmin. This is a self-regulated event as the very presence of…

Descarga: Diagnosing von Willebrand Disease: A Short History of Laboratory Milestones and Innovations, Plus Current Status, Challenges, and Solutions Archivo: eha/vonwill/STH 2014 Diagnosing von Willebrand Disease.pdf von Willebrand disease (VWD) is a disorder characterized by deficiency of, or defects in, von Willebrand factor (VWF). VWD was originally identified by Erik Adolf von Willebrand, who in…

Descarga: Genetics of inherited platelet disorders Archivo: eha/plaquetopatias/haemo 2014-Genetics of inherited platelet disorders.pdf The current review describes inherited platelet disorders, illustrates their clinical phenotype and molecular genetic defects. Platelets are the key molecules mediating haemostasis via adhesion, activation and clot formation at the site of injury. The inherited platelet disorders can be classified according to…

Descarga: Management of acquired haemophilia A Archivo: eha/inhibfact/haemo 2015-Management of acquired haemophilia A.pdf Acquired haemophilia A (AHA) is caused by autoantibody inhibitors of coagulation factor VIII (FVIII : C). Recent onset of bleeds and isolated prolongation of the activated partial thromboplastin time (aPTT) are characteristic features of the disorder. Reduced FVIII : C activity and…

Descarga: Platelet pathology and antiplatelet strategies Archivo: eha/plaquetopatias/haemo 2016 Platelet pathology and antiplatelet strategies.pdf Blood platelets were the focus of recent issues  of this Journal (1, 2). Why do these inconspicuous, small cellular fragments, derived from megakaryocytes in the bone marrow, gain so much attention both in clinical settings and basic science? Platelets contribute essentially…

Descarga: Platelet pathology and antiplatelet strategies Archivo: eha/plaquetopatias/haemo 2016 Platelet pathology and antiplatelet strategies.pdf Blood platelets were the focus of recent issues  of this Journal (1, 2). Why do these inconspicuous, small cellular fragments, derived from megakaryocytes in the bone marrow, gain so much attention both in clinical settings and basic science? Platelets contribute essentially…

Descarga: Pregnancy in Upshaw-Schulman syndrome Archivo: eha/vonwill/haemo 2013-Pregnancy in Upshaw-Schulman syndrome.pdf The Upshaw Schulman syndrome (MIM #274150) is a hereditary deficiency of the von Willebrand factor cleaving protease (ADAMTS13) due to homozygous or compound heterozygous mutations in the ADAMTS13 gene. Patients are prone to bouts of thrombotic thrombocytopenic purpura. However, disease manifestation needs a second…