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Leniolisib: Pediatric drug information

Leniolisib: Pediatric drug information
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For additional information see "Leniolisib: Drug information" and "Leniolisib: Patient drug information"

For abbreviations, symbols, and age group definitions show table
Brand Names: US
  • Joenja
Dosing: Pediatric
Activated phosphoinositide 3-kinase delta syndrome

Activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome: Children ≥12 years and Adolescents, weighing ≥45 kg: Oral: 70 mg every 12 hours.

Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.

Dosing: Kidney Impairment: Pediatric

There are no dosage adjustments provided in the manufacturer's labeling.

Dosing: Liver Impairment: Pediatric

Children ≥12 years and Adolescents, weighing ≥45 kg: Oral:

Mild impairment: There are no dosage adjustments provided in the manufacturer's labeling; has not been studied. Leniolisib is metabolized extensively (60%) by the liver; monitor closely.

Moderate to severe impairment: Use not recommended; has not been studied.

Dosing: Adult

(For additional information see "Leniolisib: Drug information")

Activated phosphoinositide 3-kinase delta syndrome

Activated phosphoinositide 3-kinase delta syndrome: Oral: Weight ≥45 kg: 70 mg twice daily, approximately every 12 hours (Ref).

Missed dose : If a dose is missed by >6 hours, skip the missed dose and administer the next dose at the usual time. If a dose is vomited ≤1 hour after administration, administer another dose as soon as possible; if vomited >1 hour after administration, do not readminister the dose (administer the next dose at the usual time).

Dosage adjustment for concomitant therapy: Significant drug interactions exist, requiring dose/frequency adjustment or avoidance. Consult drug interactions database for more information.

Dosing: Kidney Impairment: Adult

There are no dosage adjustments provided in the manufacturer's labeling.

Dosing: Liver Impairment: Adult

Mild impairment (Child-Pugh class A): There are no dosage adjustments provided in the manufacturer's labeling (has not been studied).

Moderate to severe impairment (Child-Pugh class B or C): Use is not recommended (effect on leniolisib pharmacokinetics has not been studied).

Adverse Reactions (Significant): Considerations
Neutropenia

Neutropenia has been reported with leniolisib. In a clinical study, this absolute neutrophil count (ANC) decrease did not result in infections and was considered mild and transient. No ANC <500 cells/µL or events ≥ grade 3 were reported (Rao 2023).

Transient and mild neutropenia has been reported with leniolisib. In clinical studies, an absolute neutrophil count (ANC) <500 cells/µL, events ≥ grade 3, and infection were not reported (Rao 2023).

Onset: Intermediate; nadir at day 15 with recovery by day 85 has been reported (Rao 2023).

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Adverse reactions reported in adults and adolescents.

>10%:

Dermatologic: Atopic dermatitis

Hematologic & oncologic: Neutropenia

Nervous system: Headache

Respiratory: Sinusitis

1% to 10%:

Cardiovascular: Tachycardia

Dermatologic: Alopecia

Gastrointestinal: Diarrhea

Nervous system: Fatigue

Neuromuscular & skeletal: Back pain, neck pain

Miscellaneous: Fever

Contraindications

There are no contraindications listed in the manufacturer's labeling.

Warnings/Precautions

Other warnings/precautions:

• Immunizations: Live attenuated vaccinations may be less effective when administered during leniolisib treatment.

Dosage Forms: US

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Tablet, Oral, as phosphate:

Joenja: 70 mg

Generic Equivalent Available: US

No

Pricing: US

Tablets (Joenja Oral)

70 mg (per each): $990.00

Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.

Administration: Pediatric

Oral: Administer with or without food. If patient vomits ≤1 hour after dose, readminister dose as soon as possible; if patient vomits >1 hour after dose, do not readminister dose; wait and administer next dose at scheduled time.

Missed dose: If dose missed by >6 hours, wait and take next dose at scheduled time.

Administration: Adult

Oral: Administer doses approximately every 12 hours, with or without food. If vomiting occurs ≤1 hour after administration, administer another dose as soon as possible. If vomiting occurs >1 hour after administration, do not readminister the dose; wait and administer the next dose at the usual time.

Storage/Stability

Store at 20°C to 25°C (68°F to 77°F); excursions permitted between 15°C to 30°C (59°F to 86°F); do not refrigerate. Store and dispense in original container.

Use

Treatment of activated phosphoinositide 3-kinase delta (PI3Kδ) syndrome (FDA approved in ages ≥12 years and adults).

Medication Safety Issues
Sound-alike/look-alike issues:

Joenja may be confused with Jolessa, Jorveza.

Leniolisib may be confused with alpelisib, copanlisib, duvelisib, idelalisib, lenvatinib.

Metabolism/Transport Effects

Substrate of BCRP, CYP3A4 (Major), P-glycoprotein (Minor); Note: Assignment of Major/Minor substrate status based on clinically relevant drug interaction potential;

Drug Interactions

Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the drug interactions program by clicking on the “Launch drug interactions program” link above.

Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the drug interactions program

BCRP/ABCG2 Substrates (Clinically Relevant with Inhibitors): Leniolisib may increase serum concentration of BCRP/ABCG2 Substrates (Clinically Relevant with Inhibitors). Risk X: Avoid

Clofazimine: May increase serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Risk C: Monitor

CYP1A2 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors): Leniolisib may increase serum concentration of CYP1A2 Substrates (Narrow Therapeutic Index/Sensitive with Inhibitors). Risk X: Avoid

CYP3A4 Inducers (Moderate): May decrease serum concentration of Leniolisib. Risk X: Avoid

CYP3A4 Inducers (Strong): May decrease serum concentration of Leniolisib. Risk X: Avoid

CYP3A4 Inhibitors (Moderate): May increase serum concentration of Leniolisib. Risk C: Monitor

CYP3A4 Inhibitors (Strong): May increase serum concentration of Leniolisib. Risk X: Avoid

Fusidic Acid (Systemic): May increase serum concentration of CYP3A4 Substrates (High risk with Inhibitors). Management: Consider avoiding this combination if possible. If required, monitor patients closely for increased adverse effects of the CYP3A4 substrate. Risk D: Consider Therapy Modification

Grapefruit Juice: May increase serum concentration of Leniolisib. Risk C: Monitor

OATP1B1/1B3 (SLCO1B1/1B3) Substrates (Clinically Relevant with Inhibitors): Leniolisib may increase serum concentration of OATP1B1/1B3 (SLCO1B1/1B3) Substrates (Clinically Relevant with Inhibitors). Risk X: Avoid

Vaccines (Live): Leniolisib may decrease therapeutic effects of Vaccines (Live). Risk C: Monitor

Reproductive Considerations

Evaluate pregnancy status prior to use; verify the patient is not pregnant prior to treatment initiation (in patients who could become pregnant).

Patients who could become pregnant should use highly effective contraception during therapy and for 1 week after the last leniolisib dose.

Pregnancy Considerations

Based on data from animal reproduction studies, in utero exposure to leniolisib may cause fetal harm.

Monitoring Parameters

Pregnancy test in patients who can become pregnant (prior to the initiation of therapy and as deemed clinically necessary during treatment and up to 1 week after stopping therapy); liver function tests; complete blood count with differential.

Mechanism of Action

Leniolisib is a kinase inhibitor that selectively targets phosphoinositide 3-kinase (PI3K) delta (Rao 2023). Leniolisib blocks the active binding site of PI3K delta, inhibiting the signaling pathways that lead to increased production of PIP3, hyperactivity of the downstream mTOR/AKT pathway, and dysregulation of B and T cells.

Pharmacokinetics (Adult Data Unless Noted)

Distribution: Vd: ~28.5 L.

Protein binding: 94.5%.

Metabolism: Primarily hepatic via CYP3A4, with minor contribution to metabolism by CYP3A5, CYP1A2, and CYP2D6.

Half-life elimination: Terminal: ~10 hours; Effective: ~7 hours.

Time to peak:

Children ≥12 years and Adolescents: Median: ~3 hours (range: 1 to 5 hours).

Adults: Median: ~1 hour.

Excretion: Urine: 25.5% (6.32% as unchanged drug); feces: 67%.

  1. Joenja (leniolisib) [prescribing information]. Fallavier, France: Skyepharma Production; March 2023.
  2. Rao VK, Webster S, Šedivá A, et al. A randomized, placebo-controlled phase 3 trial of the PI3Kδ inhibitor leniolisib for activated PI3Kδ syndrome. Blood. 2023;141(9):971-983. doi:10.1182/blood.2022018546 [PubMed 36399712]
  3. Refer to manufacturer's labeling.
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