[由原发性乳腺淋巴瘤复杂的Sjögren病:一个病例报告]
1Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China.
概括
本案例报告强调了Sjögren病 (SjD) 患者患有扩散性大B细胞淋巴瘤 (DLBCL) 的罕见病例,强调了在SjD中诊断非典型表现和恶性瘤时需要保持警.
科学领域:
- 类风湿病学 类风湿病学
- 在瘤学瘤学.
- 病理学 病理学 病理学
背景情况:
- 斯约格伦病 (SjD) 是一种慢性自身免疫性疾病,主要影响外分泌腺,但系统性表现和淋巴瘤风险增加是已知的并发症.
- 这个病例涉及一个67岁的女性,有40年的SjD病史,出现长期发烧,需要对源不明的发烧进行彻底的诊断检查.
研究的目的:
- 描述Sjögren病患者的诊断和治疗过程,该患者患有不寻常的并发症.
- 强调在Sjögren病患者长期发烧的差异诊断中考虑淋巴瘤的重要性.
主要方法:
- 临床评估,血清学测试 (ANA,抗SSA/Ro,抗α-fodrin),希尔默测试,胸部CT,欧洲Sjögren综合征疾病活动指数 (ESSDAI) 评分.
- 实验室检查感染,评估淋巴增殖性疾病标志物 (M蛋白,LDH,费里丁,β2-微球蛋白),骨髓吸收,PET/CT成像和乳腺损伤的组织病理学检查.
- 用R-CHOP化疗治疗已确认的扩散性大B细胞淋巴瘤 (DLBCL) 的治疗.
主要成果:
- 患者最初在低剂量皮质类固醇和DMARD上稳定,但发烧持续时间长. 尽管感染结果是负面的,但较高的标志物引起了对淋巴瘤的怀疑.
- PET/CT揭示了乳房和大腿骨中的高代谢病变;组织病理学证实非霍奇金淋巴瘤 (NHL),特别是DLBCL (GCB亚型).
- 经过R-CHOP化疗,在三次循环后实现了完全的代谢缓解,尽管患者经历了与治疗相关的骨髓抑制和肺部感染.
结论:
- 这一案例强调了在没有典型风险因素的情况下,在Sjögren病患者中,对非典型表现和恶性瘤,特别是淋巴瘤的高怀疑指数的关键需求.
- PET/CT和组织病理学是评估格伦病疑似恶性病的关键诊断工具.
- 乳腺淋巴瘤在Sjögren病中罕见的发生,需要进一步研究其发病因子,可能涉及淋巴细胞透和恶性转变.
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