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Kaposiform lymphangiomatosis—the effects of long-term treatment with sirolimus: case series study and review of the literature

IntroductionKaposiform lymphangiomatosis (KLA) is an ultrarare disease characterized by abnormal lymphatic vessel proliferation due to hyperactivation of the Rat sarcoma virus (RAS) signaling pathway, resulting in multifocal lymphatic malformations (LMs) that can potentially involve multiple organ s...

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Hoofdauteurs: Magdalena Bilny-Paluch, Piotr Piekarczyk, Katarzyna Błasińska, Małgorzata Szołkowska, Mateusz Polaczek, Elżbieta Radzikowska
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Frontiers Media S.A. 2026-04-01
Reeks:Frontiers in Medicine
Onderwerpen:
complex lymphatic anomalies
kaposiform lymphangiomatosis
lymphatic malformation
PI3K/AKT/mTOR pathway
rapamycin
sirolimus
Online toegang:https://www.frontiersin.org/articles/10.3389/fmed.2026.1705297/full
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