Fresh frozen plasma and cryoprecipitate Fresh frozen plasma and cryoprecipitate are the mainstay of therapy for<unk> , but carry risk related to transfusion. Factor<unk>  concentrates Two commercially produced factor<unk>  concentrates are currently available in Europe, one manufactured by Bio Products Laboratory (BPL) and only available in the United Kingdom. The other, Fibrogammin-P, is produced by Beringwerke of Germany. In the U.S. FXIII concentrate is only available under the Federal Drug Administration's Investigational New Drug (IND) Program, or through clinical trial. Recombinant factor<unk>  Recombinant factor<unk>  (rFXIII) is the only drug alternative to receiving blood transfusions, the traditional treatment for<unk> . Novo Nordisk’s rFXIII, catridecacog, was approved by the US Food and Drug Administration in 2014. Although it is a recombinant protein, rFXIII subunit A is identical in structure and function to the A subunit of factor<unk>  naturally produced in the body by healthy individuals. These patients need exogenous subunit A of factor<unk>  since they have a mutation which prevents production of the A subunit. However, since the B-subunit is located on a separate chromosome, factor<unk>  deficient patients actually produce the B-subunit normally. When these two subunits interact in the plasma, the enzyme is activated and can act within the clotting cascade. rFXIII acts by inhibiting fibrinolysis factors which enzymatically cleave the fibrin matrix, leading to the ultimate formation of clots. rFXIII is synthetically bio-engineered through a yeast expression system and administered intravenously. In clinical trials, the drug was administered once every four weeks or administered on-demand in order to treat bleeding episodes. The introduction of rFXIII as a treatment for<unk>  eliminates the risk of pathogenic infection present in plasma-based treatments. rFXIII treatment would also not be dependent on blood donations, consequently increasing availability and product quality. One of the biggest fears in developing rFXIII was that the body would mount an immune-response to the protein; however, several safety and pharmacokinetics studies have reported no immunogenic response to rFXIII or associated yeast products.
Factor XIII deficiency
Factor VII deficiency
Factor XII deficiency
Factor I deficiency
Factor X deficiency
Protein C deficiency
COAT platelet defect
Antithrombin III deficiency