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Article

Pathogenic myeloid phenotypes drive disease pathology in a novel human neurohistiocytosis model.

Blood - 23 Jul 2026

Rajamanickam Shivakumar, Trescott Samantha, Mak Samantha, Warden Anna S, Wilpitz Amanda M, Xia Bing, Nguyen Celina, Ding Hilda, Picarsic Jennifer, Glass Christopher K, Allen Carl E, Coufal Nicole G

Abstract excerpt

ABSTRACT: Innate immunity is increasingly recognized as a driver of neurodegeneration, although pathogenic mechanisms are incompletely understood. Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplastic disorder caused by activating somatic mutations in MAPK pathway genes, most commonly BRAFV600E, in myeloid precursors. A subset of patients with LCH develop progressive neurodegeneration...

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Identifiers

PMID: 41915769
DOI: 10.1182/blood.2025032207
PMCID: PMC13487487

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