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PI3K-delta syndrome; PASLI disease
Pathology:
- hyperactive signaling of PI3K-delta
Clinical manifestations:
- infections
- lymphocyte proliferation (lymphocytosis)
- autoimmunity
- enteropathy
- bronchiectasis
- increased risk of lymphoma
- early mortality
Management:
- leniolisib (Joenja) FDA-approved
General
immunologic disease
syndrome
References
- Rao VK et al
A randomized, placebo-controlled phase 3 trial of the PI3Kdelta inhibitor
leniolisib for activated PI3Kdelta syndrome
Blood. 2023 Mar 2;141(9):971-983
PMID: 36399712 PMCID: PMC10163280 Free PMC article