ArticleAnnals of hematology2025
Diffuse Large B-cell lymphoma in a Ras-Associated Autoimmune Leukoproliferative Disorder (RALD).
Article in Annals of hematology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
RAS genes are among the most mutated genes in human cancers, with KRAS G12C now being targetable by the specific inhibitor Sotorasib. Ras-associated autoimmune leukoproliferative disorder (RALD) is a rare condition characterized by somatic RAS mutations and hyperactive MAPK signaling. RALD typically affects pediatric patients and is generally considered a benign condition. We report a unique case of RALD in a 76-year-old patient, who is the oldest individual with this disorder to date. This patient also developed lymphoma, marking the fourth known case of malignancy associated with RALD. Additionally, a novel fusion, RHOH::PDCD1LG2 (PD-L2), was identified in the lymphoma, alongside the KRAS mutation. This fusion likely contributes to tumorigenesis through immune evasion.
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