factor XIII A-subunit, recombinant

Action and Spectrum

Actions and Spectrum: 

factor XIII A-subunit recombinant is a medication used to treat congenital factor XIII deficiency, a rare blood disorder characterized by abnormal bleeding.  

The mechanism of action of factor XIII A-subunit recombinant is to replace the missing or deficient factor XIII in the blood. factor XIII is a clotting factor that plays a crucial role in the final stages of blood clot formation, where it stabilizes the clot by crosslinking fibrin, a protein that forms the backbone of the clot. 

factor XIII A-subunit recombinant is a laboratory-produced version of the A-subunit of factor XIII, the catalytic subunit responsible for crosslinking fibrin. When administered, the factor XIII A-subunit recombinant binds to the deficient factor XIII B-subunit, forming a functional factor XIII complex that can crosslink fibrin and stabilize blood clots. 

The spectrum of activity of factor XIII A-subunit recombinant is limited to treating congenital factor XIII deficiency. It is inadequate for treating other bleeding disorders, such as hemophilia, involving different clotting factors. 

Drug Interaction

Adverse Reaction

Frequency not defined 

≥1% 

Extremity pain 

Increased fibrin D dimer levels 

Headache 

Injection site pain 

<1% 

Antibody formation 

Black Box Warning

Contraindication / Caution

Contraindications/caution: 

Contraindications: 

• factor XIII A-subunit recombinant is a medication used to treat congenital factor XIII deficiency. As with any medication, specific contraindications should be considered before administering it. 

• The use of factor XIII A-subunit, recombinant, is contraindicated in patients with a known hypersensitivity to any of the components of the product. It should also not be used in patients with a history of allergic reactions to other recombinant human proteins. 

• Additionally, using factor XIII A-subunit, recombinant should be avoided in patients who have experienced thrombotic events (blood clots) or have a known increased risk of thrombosis (abnormal blood clotting). Factor XIII A-subunit recombinant can increase the risk of thrombosis, especially if administered at high doses. 

• Furthermore, factor XIII A-subunit recombinant is not recommended in pregnant or breastfeeding women unless necessary, as its effects on fetal development and milk production are poorly understood. 

Caution: 

• factor XIII A-subunit recombinant is a medication used to treat congenital factor XIII deficiency. While it is generally well-tolerated, specific cautions should be considered before administering it. 
• One caution is that factor XIII A-subunit recombinant can increase the risk of thrombosis (abnormal blood clotting), especially if administered at high doses. Therefore, caution should be exercised when administering the medication to patients with a known increased risk of thromboses, such as those with a history of deep vein thrombosis (DVT), pulmonary embolism (PE), or thrombotic microangiopathy (TMA). 
• Another caution is that factor XIII A-subunit, recombinant may interfere with coagulation tests, such as the prothrombin time (PT) and activated partial thromboplastin time (aPTT), which are commonly used to monitor blood clotting function. This can lead to inaccurate test results and may affect the ability to monitor and adjust anticoagulant therapy. Therefore, coagulation tests should be performed before and after administering factor XIII A-subunit, recombinant, using specialized assays not affected by the medication. 

Pregnancy / Lactation

Pregnancy consideration: Insufficient data available 

Lactation: Excretion of the drug in human breast milk is unknown 

Pregnancy category: 

Category A: well-controlled and Satisfactory studies show no risk to the fetus in the first or later trimester.   

Category B: there was no evidence of risk to the fetus in animal studies, and there were not enough studies on pregnant women. 

Category C: there was evidence of risk of adverse effects in animal reproduction studies, and no adequate evidence in human studies must take care of potential risks in pregnant women.    

Category D: adequate data with sufficient evidence of human fetal risk from various platforms, but despite the potential risk, and used only in emergency cases for potential benefits.    

Category X: Drugs listed in this category outweigh the risks over benefits. Hence these categories of drugs need to be avoided by pregnant women.    

Category N: There is no data available for the drug under this category 

Pharmacology

Pharmacology: 

factor XIII A-subunit, recombinant medication is used to treat congenital factor XIII deficiency. factor XIII is a clotting factor critical in the final stages of blood coagulation. factor XIII A-subunit, recombinant works by replacing the missing or deficient factor XIII in patients with congenital factor XIII deficiency. 

Once administered, factor XIII A-subunit recombinant combines with factor XIII B-subunit to form the active transglutaminase enzyme, which crosslinks fibrin, stabilizing the blood clot. Crosslinking helps strengthen the clot and prevent it from breaking down prematurely. 

Pharmacodynamics: 

The pharmacodynamics of recombinant factor XIIIA-subunit involves its ability to enhance clot strength and stability by crosslinking fibrin, a protein involved in blood clotting. Recombinant factor XIIIA-subunit is administered as an intravenous injection and works by forming crosslinks between fibrin molecules to create a more robust and stable clot. 

Recombinant factor XIIIA-subunit has a long half-life of about 10-14 days, meaning it remains active in the body for an extended period. This allows for less frequent dosing, typically every four weeks, and provides sustained clotting activity for people with Factor XIII deficiency. 

Pharmacokinetics: 

Absorption 

Recombinant factor XIII A-subunit is administered as an intravenous injection and is directly introduced into the bloodstream. As a result, absorption is not a factor that needs to be considered. 

Distribution 

factor XIII A-subunit, recombinant, is a large protein molecule distributed throughout the body via the bloodstream. The distribution is limited to the extracellular space, and the protein does not cross the blood-brain barrier. The volume of distribution is estimated to be approximately 0.1 L/kg. 

Metabolism 

factor XIII A-subunit, recombinant, is primarily metabolized by proteolysis, a process in which the protein is broken down into smaller peptides and amino acids. No known metabolic pathways significantly impact the recombinant clearance of factor XIII A-subunit. 

Elimination and Excretion 

The primary route of recombinant excretion for factor XIII A-subunit is via the kidneys. However, due to its considerable molecular weight, recombinant factor XIII A-subunit clearance is slow, with an elimination half-life of approximately 10-14 days. It is also possible for the protein to be cleared via the reticuloendothelial system. 

Adminstartion

Administration: 

factor XIII A-subunit, recombinant, is administered as an intravenous injection by a healthcare professional. The medication is supplied as a powder that needs to be reconstituted with sterile water for injection before administration. The specific dose and frequency of factor XIII A-subunit recombinant administration will depend on the individual patient’s needs and the severity of their factor XIII deficiency. 

The recommended dose for preventing bleeding in adults and children with congenital factor XIII A-subunit deficiency is 35 IU/kg body weight, administered intravenously every 28 ± 4 days. The initial dose for on-demand treatment of bleeding episodes is 60 IU/kg body weight, followed by a maintenance dose of 10-20 IU/kg body weight every 28 ± 4 days until complete wound healing or cessation of bleeding. 

factor XIII A-subunit, recombinant, should be administered slowly over several minutes, and patients should be monitored for any signs of an allergic reaction. The medication should not be mixed with other medications or solutions. 

Patient Information Leaflet

Patient information leaflet 

Generic Name: factor XIII A-subunit, recombinant 

Why do we use factor XIII A-subunit, recombinant? 

factor XIII A-subunit, recombinant, is a medication used to treat people with factor XIII deficiency, a rare bleeding disorder that affects the blood’s ability to clot properly. factor XIII deficiency is an inherited disorder that results in decreased levels of factor XIII in the blood. Without enough factor XIII, the blood cannot form stable clots, leading to increased bleeding and poor wound healing. 

• The primary use of factor XIII A-subunit, recombinant, is for the prevention and treatment of bleeding in people with congenital factor XIII deficiency. The medication works by enhancing the strength and stability of blood clots, reducing the risk of bleeding, and promoting healing. 
• factor XIII A-subunit, recombinant, may also be used to manage bleeding in people with acquired factor XIII deficiency, which can occur due to conditions such as liver disease, leukemia, or lymphoma. However, its effectiveness in these conditions has not been thoroughly evaluated, and its use in acquired factor XIII deficiency is considered off-label.