Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma is rare among lung neoplasia cases, representing only 0.5%-1% of newly diagnosed primary lung lymphoma. MALT lymphoma with relapsed refractory and malignant transformation is highly heterogeneous and consensus therapy remains undetermined. We report a 55 year-old woman with a 3 year history of primary pulmonary MALT lymphoma confined to the lung presenting with massive pleural effusion. After two cycles of R-CHOP and six cycles of R2-CHOP, pleural effusion disappeared but the pulmonary mass remained persistent. Second-line therapies R2-GemOx failed to make any substantial improvement. Core-needle puncture biopsy of the pulmonary mass was obtained and pathological testing revealed transformed diffuse large B-cell lymphoma of germinal center B-cell subtype. Next-generation sequencing confirmed BN2 subtype. The mass showed no reduction after three cycles of R-MINE, following which the BTK inhibitor ibrutinib was administered to this patient. Unfortunately, after two months of ibrutinib treatment, the patient rapidly developed an enlarged mass and hyperprogressive disease, to which she subsequently succumbed.

中文翻译：

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来自原发性肺黏膜相关淋巴组织淋巴瘤的转化弥漫性大 B 细胞淋巴瘤患者的依鲁替尼耐药。

黏膜相关淋巴组织 (MALT) 淋巴瘤的结外边缘区淋巴瘤在肺肿瘤病例中很少见，仅占新诊断的原发性肺淋巴瘤的 0.5%-1%。具有复发难治性和恶性转化的 MALT 淋巴瘤具有高度异质性，共识治疗仍未确定。我们报告了一名 55 岁女性，她有 3 年的原发性肺 MALT 淋巴瘤病史，表现为大量胸腔积液。两个周期的 R-CHOP 和六个周期的 R2-CHOP 后，胸腔积液消失，但肺部肿块仍然存在。二线疗法 R2-GemOx 未能取得任何实质性的改善。获得肺肿块的核心针穿刺活检，病理检查显示转化为生发中心 B 细胞亚型的弥漫性大 B 细胞淋巴瘤。下一代测序证实了 BN2 亚型。在 R-MINE 的三个周期后，肿块显示没有减少，此后给该患者服用 BTK 抑制剂依鲁替尼。不幸的是，在接受依鲁替尼治疗两个月后，患者迅速出现肿块肿大和过度进展的疾病，随后死亡。