Advances in recombinant technology and knowledge about coagulation factor VIII (FVIII) are building a platform for new therapeutic options in patients with hemophilia A. The development of turoctocog alfa, a novel, high-purity, third-generation, B-domain truncated recombinant FVIII, has been produced and formulated without the use of animal-derived or human serum-derived components, in the wake...

The classification of factor VIII deficiency, generally used based on plasma levels of factor VIII, consists of severe (<1% normal factor VIII activity), moderate (1% to 4% factor VIII activity), or mild (5% to 25% factor VIII activity). A recent communication described four individuals bearing identical factor VIII mutations. This resulted in a severe bleeding disorder in two patients who carr...

INTRODUCTION Starting from the point that the chronic kidney disease (CKD) is chronic, inflammatory and hypercoagulable state characterized by an increase in procoagulant and inflammatory markers high cardiovascular morbidity and mortality in these patients could be explained. AIM The aim of the research was to monitor inflammatory markers and procoagulants in various stages of kidney disease...

Conventional clotting factor concentrates have, until recently. been “of intermediate purity.” containing <1 % of the coagulation factor, and >99% extraneous plasma proteins such as fibrinogen, fibronectin. gamma globulins. and traces of many others. We report here the results of a new factor VIII concentrate that is purified from human plasma using a mouse monoclonal antibody to factor Vlll:vW...

The factor VIII coagulant activity (FVIII:C), factor VIII related antigen (FVIII:RAG), and factor VIII ristocetin cofactor activity (FVIII:RCF) was determined in the cord blood samples of 30 healthy term newborns. Sodium citrate anticoagulant, cold, and a proteolytic inhibitor were used in sample processing. All three factor VIII activities were elevated in infants compared to adults; additiona...

Haemophilia A is a rare inherited bleeding disorder characterized by an inability of the blood to clot normally. Patients can experience spontaneous or trauma-induced joint and soft tissue bleeding and must keep coagulation factor VIII (FVIII) accessible at all times; thus, FVIII product storage and stability are critical. Our primary objective was to assess haemophilia A patients' and caregive...

Perfusionists should be able to verify diagnosis and recognize the forms of von Wille brand disease (vWD) which may require specific coagulation management during open heart procedures. von Willebrand factor (vWF) plays two distinct and important roles in clot formation subsequent to vascular injury. First, it promotes platelet binding to subendothelial surfaces and other platelets (platelet ag...

many investigations have proved relations between abo blood groups with some diseases and factor viii and von willebrand level in plasma. in this study we investigated a relation between abo blood groups and factor viii and ix inhibitors in 102 patients with haemophilia a and 48 patients with haemophilia b. the assay of inhibitor was done by bethesda method. there were no relation between abo b...

Removal of blood-based additives from recombinant clotting factor concentrates continues to be advocated by the hemophilia community due to the history of infectious disease transmission with previous blood-derived clotting factor concentrates. In 2003, octocog-alpha, antihemophilic factor (recombinant), plasma/albumin-free method (ADVATE) was introduced, providing the first third-generation re...

We present a case of a 79-year-old male who presented with retroperitoneal hematoma a week after motor vehicle accident. Prior history and family history of bleeding were nonsignificant. His activated partial thromboplastin time was found to be prolonged in the emergency department. Further workup with coagulation studies showed decreased factor VIII, vWF antigen, and vWF:ristocetin cofactor as...