Protein Z (PZ) is a vitamin K-dependent factor identified in human plasma in 1984 characterized by an homology with other vitamin K-dependent factors (factor VII, IX, X, protein C). In contrast to these factors, PZ does not possess any enzymatic activity but is involved as a cofactor in the down-regulation of coagulation by forming a complex with the protein Z-dependent protease inhibitor (ZPI)...

Patients with coumarin induced skin necrosis presents with painful purpura and erythematous bluish swelling of the skin complicated by blister formation, hemorrhage and immanent gangrene. Coumarin induced hemorrhagic skin necrosis occurs within one week after initiation of coumarin treatment at time of prolonged INR caused by an imbalance within the vitamin K dependent anticoagulant and procoag...

Coagulaon factor XIII gene, protein structure and funcon Coagulaon factor XIII (FXIII) is a tetrameric (FXIII- A2B2) pro-transglutaminase enzyme with an essenal role in the final stage of coagulaon cascade by cross linking the fibrin monomers and stabilizing the fibrin clot. Congenital FXIII deficiency is a rare bleeding disorder, with an autosomal recessive trait inheritance, and a fre...

This review summarizes current data on the pathomechanisms and new genetic findings of combined factor V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined factor V and VIII deficiency (F5F8D) was reported b...

The presence of gene lesions in coagulation factor X (FX, Stuart factor) was investigated in patients with FX deficiency or an FX abnormality (FX Friuli). The proposita had a heterozygous partial deletion of the FX gene with severe deficiency of FX activity and antigen. The lesion, which was inherited from her mother, removes the 3' portion of the gene coding for the catalytic domain of the fac...

Hemophilia is an X-linked recessive bleeding disorder caused by deficiency of FVIII (hemophilia A) or FIX (hemophilia B). Mutations in the FVIII or FIX genes, both located on the long arm of the X chromosome, are detectable in the majority of cases of hemophilia A or B, respectively. During normal hemostasis, FVIII and FIX form an enzymatic complex; thus, deficiency of either protein leads to a...

background: factor vii concentrates are used in patients with congenital or acquired factor vii deficiency or treatment of hemophilia patients with inhibitors. in this research, immunoaffinity chromatography was used to purify factor vii from prothrombin complex (prothrombin-proconvertin-stuart factor-antihemophilic factor b or ppsb) which contains coagulation factors ii, vii, ix and x. the aim...