Bone marrow transplantation (BMT) is nowadays used in various hematological disorders including leukemias. Hemophilia A is sex linked bleeding disorder in which there are various genetic abnormalities in factor VIII gene. Among various hematological disorders, bleeding disorders mainly hemophilia in now widely treated using plasma derived and recombinant factor VIII concentrates. Day to day tra...

background bone marrow transplantation (bmt)is nowadays used in various hematological disorders including leukemias. hemophilia a & b are sex linked bleeding disorders in which there are various genetic abnormalities in factor viii & ix genes. among various hematological disorders, bleeding disorders mainly hemophilia in now widely treated using plasma derived and recombinant factor viii & ix c...

Fulminant hepatic failure (FHF) in humans produces a bleeding diathesis due in large part to a reduction in the biosynthesis of liver-derived coagulation factors. Remarkably, factor VIII procoagulant activity is elevated in most of these patients despite widespread liver cell death. FHF can be modeled in mice by administration of azoxymethane, the active ingredient found in cycad palm nuts. We ...

Factor VIII (fVIII) is a serum protein in the coagulation cascade that nucleates the assembly of a membrane-bound protease complex on the surface of activated platelets at the site of a vascular injury. Hemophilia A is caused by a variety of mutations in the factor VIII gene and typically requires replacement therapy with purified protein. We have determined the structure of a fully active, rec...

Normal human plasma contains a complex of two proteins that are important in hemostasis and coagulation. The factor VIII procoagulant protein (antihemophilic factor) and the factor VIll-related protein (von Willebrand factor) are under separate genetic control, have distinct biochemical and immunologic properties, and have unique and essential T HE IMPORTANCE of factor VIII in hemostasis and bl...

The development of inhibitory 'allo' antibodies to a deficient coagulation factor is arguably now the most severe and important complication of clotting factor concentrate exposure in haemophilia and other congenital coagulation disorders. Furthermore, development of an inhibitor to the factor VIII or factor IX transgene product remains a significant concern in gene therapy protocols for haemop...

Methylation, CpG island, promoter, intron 1 Haemophilia A is the most common X-linked inherited coagulation disorder caused by a deficiency of the factor VIII protein (FVIII). A plethora of different mutations in the factor VIII gene (F8) have been identified as causative for this bleeding disease including a few promoter mutations. However, in approximately 2-5% of all haemophilic patients, th...

Immunologic methods were employed in an attempt to identify a potent procoagulant present in homogenates of human skin fibroblasts cultured in vitro. The activity of this procoagulant was restricted to the early stages of coagulation and was heretofore considered to be due to tissue factor (tissue thromboplastin, factor Ill) either alone or in combination with one or more of the first-stage coa...

Normal human plasma contains a complex of two proteins that are important in hemostasis and coagulation. The factor VIII procoagulant protein (antihemophilic factor) and the factor VIll-related protein (von Willebrand factor) are under separate genetic control, have distinct biochemical and immunologic properties, and have unique and essential T HE IMPORTANCE of factor VIII in hemostasis and bl...

Electroconvulsive therapy (ECT) is used for medication-resistant and life-threatening mental disorders, and therefore it occupies an important position in psychiatric treatment. ECT reportedly increases intracranial pressure and is suspected of increasing the risk of intracranial hemorrhage, especially in patients with hemorrhagic diseases such as hemophilia. A decrease in or loss of blood coag...