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Identifying and targeting pathogenic PI3K/AKT/mTOR signaling in IL-6-blockade-refractory idiopathic multicentric Castleman disease.

著者 Fajgenbaum DC , Langan RA , Sada Japp A , Partridge HL , Pierson SK , Singh A , Arenas DJ , Ruth JR , Nabel CS , Stone K , Okumura M , Schwarer A , Jose FF , Hamerschlak N , Wertheim GB , Jordan MB , Cohen AD , Krymskaya V , Rubenstein A , Betts MR , Kamb
J Clin Invest.2019 Aug 13 ; 130():.
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Idiopathic multicentric Castleman disease (iMCD) is a hematologic illness involving cytokine-induced lymphoproliferation, systemic inflammation, cytopenias, and life-threatening multi-organ dysfunction. The molecular underpinnings of interleukin-6(IL-6)-blockade refractory patients remain unknown; no targeted therapies exist. In this study, we searched for therapeutic targets in IL-6-blockade refractory iMCD patients with the thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin myelofibrosis, renal dysfunction, organomegaly (TAFRO) clinical subtype.
PMID: 31408438 [PubMed - in process]
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