IDELVION
IDELVION Uses, Dosage, Side Effects, Food Interaction and all others data.
IDELVION (rIX-RFP) is a recombinant fusion protein that links a recombinant coagulation factor IX (rFIX) with a recombinant human albumin (rAlbumin). It was developed by CSL Behring Canada, Inc and approved by Health Canada on April 26, 2017. It was also approved by FDA and EMA in 2016. It is currently marketed in the forms of 250, 500, 1000 and 2000 IU/vial.
Clinical trials with rIX-RFP in patients with moderately to severe hemophilia B demonstrated a lower annualized spontaneous, total and joint bleeding rates. It was also efficient against bleeding episodes and maintenance of hemostasis in the perioperative setting when compared with on-demand treatment. The administration of rIX-RFP presented no reports of inhibitor development.
| Trade Name | IDELVION |
| Generic | Albutrepenonacog alfa |
| Albutrepenonacog alfa Other Names | Albutrepenonacog alfa, Coagulation factor IX (recombinant), albumin fusion protein, Coagulation Factor IX (Recombinant), Albumin Fusion Protein (rIX-FP) |
| Type | Injection, Intravenous Kit, Intravenous Powder For Injection, Intravenous |
| Formula | C5077N7846O1588PS67 |
| Weight | 125000.0 Da |
| Protein binding | This pharmacokinetic value is not relevant as this drug is part of the plasma proteins. |
| Groups | Approved |
| Therapeutic Class | |
| Manufacturer | CSL Behring UK Limited |
| Available Country | United Kingdom, United States, |
| Last Updated: | September 19, 2023 at 7:00 am |
Uses
IDELVION is a recombinant Factor IX fused to recombinant human albumin used to treat hemophilia B.
Under the EMA and FDA, rIX-RFP is indicated in the treatment of hemophilia B. For Health Canada, rIX-FRP is also indicated to prevent or reduce bleeding episodes.
Hemophilia B is the second most common type of hemophilia. It is a rare inherited bleeding disorder caused by reduced or absent levels of factor IX (FIX). The FIX is a vitamin K-dependent plasma protease that when activated is involved in the blood coagulation cascade. The hemophilia B is caused by mutations in the FIX gene which can cause different phenotypes. The severe form is characterized by the presence of spontaneous and recurring bleeds into the joints and muscles and excessive bleeding after trauma or surgery.
IDELVION is also used to associated treatment for these conditions: Perioperative Blood Loss, Bleeding episodes
How IDELVION works
The current therapies against hemophilia B are hampered by the short half-life of the replacement FIX therapy. Thus, to solve this problem, in rIX-RFP there is the fusion of rFIX with rAlbumin which presents a much longer half-life and it does not present interactions with the immune system.
The administration of rIX-RFP increases the plasma concentration of FIX, thus addressing the coagulation deficiency of the patient. rIX-RFP is able to circulate in the plasma as an intact zymogen thanks to the pH-dependent binding to FcRn which is a normal protection pathway from lysosomal degradation of albumin. When the FIX is needed, rAlbumin is cleaved by the same proteases that activate the FIX.
Toxicity
rIX-RFP is very well tolerated.Mutaginicity trials were performed and they confirmed an absent mutagenic potential.Fertility studies have not been performed. Developmental studies are not of major importance as there is a very low rate of incidence of hemophilia B in females.
Food Interaction
No interactions found.Volume of Distribution
The reported volume of distribution for rIX-RFP according to phase I/II and III clinical trials is 95 ml/kg.
Elimination Route
rIX-RFP absorption is very rapid as it is directly administered intravenously. In clinical trials, the maximum plasma concentration, area under the curve and mean residence time are reported to be approximately 55 IU/dL, 5500 IU.h/dL and 125 hours respectively.
Half Life
The fusion of the rFIX with rAlbumin prolongs the elimination half-life of rIX-RFP in the circulation. The reported half-life in clinical trials is 92 hours.
Clearance
In clinical trials, the weight-adjusted clearance in children and adults is reported to be 1.1 and 0.9 ml/h/kg.
Elimination Route
rIX-RFP is mainly eliminated in the urine. In preclinical studies, the distribution of urine and feces 240 hours post administration corresponded to 72.9% and 4.3% of the administered dose respectively. The elimination on the first 24 hours in urine and feces only corresponded to the 39.9% and 0.92% of the dose.
Innovators Monograph
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