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<p>RAS genes are among the most mutated genes in human cancers, with <i>KRAS</i> G12C now being targetable by the specific inhibitor Sotorasib. Ras-associated autoimmune leukoproliferative disorder (RALD) is a rare condition characterized by somatic <i>RAS</i> 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, <i>RHOH::PDCD1LG2 (PD-L2)</i>, was identified in the lymphoma, alongside the <i>KRAS</i> mutation. This fusion likely contributes to tumorigenesis through immune evasion.</p>

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Diffuse Large B-cell lymphoma in a Ras-Associated Autoimmune Leukoproliferative Disorder (RALD)

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年10月20日

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  • Chao Sun,
  • Lina Zhao,
  • Jun Xia,
  • Kai Wang,
  • Sujuan Zhou,
  • Lingfeng Liu,
  • Shudong Yang,
  • Yongqin Cao,
  • Zhiheng Li,
  • Xin Zhou,
  • Sheng Xiao

摘要

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.