常见的可变免疫缺陷障碍 (CVID) 中的胃肠道疾病:病理学模式,诊断线索和病理学家和胃肠病学家的陷
Lars Velthof1, Jeroen Geldof2, Marie Truyens2,3
1Department of Pathology, Ghent University Hospital, Ghent University, 9000 Ghent, Belgium.
Journal of clinical medicine
|January 25, 2025
概括
诊断常见变性免疫缺陷障碍 (CVID) 是具有挑战性的,因为它具有模仿其他疾病的多种胃肠道症状. 在肠道活检中识别微妙的组织病理线索对于准确的CVID诊断至关重要.
科学领域:
- 胃肠病学 胃肠病学
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 胃肠道 (GI) 疾病是常见的可变免疫缺陷障碍 (CVID) 致病的重要原因.
- 胃肠道表现的CVID往往模仿其他疾病,如乳病和炎症性肠病 (IBD),使诊断复杂化.
- 准确的CVID诊断依赖于在肠道活检中识别微妙的组织病理指标.
研究的目的:
- 为了提高对肠道活检中CVID的组织病理线索的认识.
- 为了突出诊断陷病理学家和胃肠病学家在评估CVID时.
- 通过了解其多样化的胃肠道表现来提高CVID的诊断准确性.
主要方法:
- 对63个肠道活检 (十二指肠,阴道肠,结肠肠) 和31年来7名CVID患者病史的综述.
- 组织学特征的分析,包括炎症,淋巴细胞,血细胞,淋巴细胞增生,密室/状结构,亚皮质原蛋白,亡,颗粒瘤和感染.
- 审查来自41个病理学请求的临床信息,以评估对CVID的认识.
主要成果:
- 组织学模式各不相同,模仿IBD,乳病,移植与宿主疾病 (GVHD),淋巴细胞喷发/结肠炎和质结肠炎.
- 在一些患者中观察到重叠和时间变化的组织学模式.
- 在病理学请求中指出,关于已知或疑似CVID的临床信息不足.
结论:
- 肠道中CVID的各种临床和组织学特征往往模仿其他肠道疾病,强调需要全面的临床数据.
- 非典型的组织学特征,重叠/变化的模式,以及肠道活检中的焦点血细胞稀缺性可能表明CVID.
- 提高对CVID异质呈现和组织病理学指标的认识,对于改善诊断结果至关重要.
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