leniolisib

Therapeutic Classification: none assigned

Pharmacologic Classification: kinase inhibitors

Indications ⬆ ⬇

REMS

Activated phosphoinositide 3-kinase delta syndrome.

Action ⬆ ⬇

Acts as an inhibitor of phosphatidylinositol 3-kinase-delta, thereby 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.

Therapeutic effects:

Reduction in lymphadenopathy.

Pharmacokinetics ⬆ ⬇

Absorption: Unknown.

Distribution: Well distributed to tissues.

Metabolism/Excretion: Primarily metabolized by the liver via the CYP3A4 isoenzyme, and minor contribution by the CYP3A5, CYP1A2, and CYP2D6 isoenzymes. Primarily excreted in the feces (67%), with 25.5% excreted in the urine.

Half-Life: 10 hr.

Time/Action Profile ⬆ ⬇

(plasma concentrations)

ROUTE	ONSET	PEAK	DURATION
PO	rapid	1 hr	12 hr

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Moderate or severe hepatic impairment;
OB: Pregnancy;
Lactation: Lactation.

Use Cautiously in:

Rep: Women of reproductive potential;
Pedi: Children <12 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: tachycardia

Derm: alopecia, atopic dermatitis

EENT: sinusitis

GI: diarrhea

Hemat: neutropenia

MS: pain

Neuro: fatigue, headache

Misc: fever

Interactions ⬆ ⬇

Drug-drug:

Strong CYP3A4 inhibitors, including itraconazole, may ↑ levels and the risk of toxicity; avoid concurrent use.
Strong CYP3A4 inducers, including rifampin, and moderate CYP3A4 inducers, including efavirenz, may ↓ levels and effectiveness; avoid concurrent use.
May ↑ levels and risk of toxicity of CYP1A2 substrates.
May ↑ levels and risk of toxicity of breast cancer resistance protein substrates, OATP1B1 substrates, and OATP1B3 substrates.
May ↓ the effectiveness of live vaccines.

Route/Dosage ⬆ ⬇

PO (Adults and Children ≥12 yr and ≥45 kg): 70 mg twice daily.

Availability ⬆ ⬇

Assessment ⬆ ⬇

Monitor for tachycardia, headache, sinusitis, and atopic dermatitis periodically throughout therapy.

Lab Test Considerations:

Verify negative pregnancy test before starting therapy.
Monitor liver enzymes at baseline.
Monitor CBC with differential during therapy May cause mild neutropenia.

Implementation ⬆ ⬇

PO: Administer without regard to food.

Patient/Family Teaching ⬆ ⬇

Explain purpose and side effects of medication to patient. Advise patient to read Patient Information before starting therapy.
Advise patient that if a dose is missed by >6 hr or is vomited >1 hr after dose, wait and take at the next scheduled time. If patient vomits ≤1 hr after dose, retake as soon as possible.
Inform patient that live-attenuated vaccines may be less effective when given during therapy with leniolisib.
Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Rep: May cause fetal harm. Advise women of reproductive potential to use highly effective method of contraception and not to breastfeed during therapy and for 1 week after the last dose. Advise patient to notify health care professional if pregnancy planned or suspected or if breastfeeding.

Evaluation/Desired Outcomes ⬆ ⬇

Reduction in lymphadenopathy.

US Brand Names ⬆ ⬇

Code ⬆

NDC Code

71274- Pharming Healthcare Inc.
- 71274-170- Joenja
  - 71274-170-60- 1 BOTTLE, PLASTIC in 1 CARTON (71274-170-60) / 60 TABLET, FILM COATED in 1 BOTTLE, PLASTIC

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Pronunciation ⬇

Classifications ⬆ ⬇