皮肤淋巴瘤和淋巴扩散性疾病的分类:来自EORTC皮肤淋巴瘤组织病理学小组的更新
W Kempf1,2, C Mitteldorf3, L Cerroni4
1Kempf und Pfaltz Histologische Diagnostik, Zurich, Switzerland.
概括
第五次世卫组织和国际共识分类更新了原发性皮肤淋巴瘤的诊断,识别了新的实体,并重新分类了其他类型,如CD8+状T细胞淋巴瘤. 这些分类强调整合各种数据以进行准确的诊断.
科学领域:
- 血液学和瘤学研究
- 皮肤病学 皮肤病学
- 病理学 病理学 病理学
背景情况:
- 初级皮肤淋巴瘤和淋巴扩散性疾病 (LPD) 的分类是动态的,随着新的临床,病理和分子见解而演变.
- 最近两个主要的分类,即世界卫生组织分类第五版和国际共识分类 (ICC),影响了对这些疾病的理解.
研究的目的:
- 与以前的世卫组织指南相比,提供了世卫组织第五次分类和ICC中引入的重大变化的概述.
- 突出不同原发性皮肤淋巴瘤和LPD的分类和识别中的关键修订.
主要方法:
- 对世卫组织第五次分类和ICC与以前的世卫组织分类进行比较分析.
- 审查新认可的实体和现有淋巴瘤类型的重新分类.
- 检查最新的世卫组织分类中的诊断标准和范围扩展.
主要成果:
- 这两种分类都将CD8+状T细胞淋巴瘤降级为CD8+状T细胞LPD.
- 新确定的实体包括原发性皮肤小或中型CD4+T细胞LPD,原发性皮肤玛/三角形T细胞淋巴瘤,原发性皮肤CD8+侵袭性表皮热细胞毒性T细胞淋巴瘤,以及艾普斯坦-巴尔病毒阳性粘膜皮肤.
- 主要皮肤边缘区域B细胞克隆性瘤被认为是惰性的,WHO和ICC之间有不同的术语 (淋巴瘤与LPD).
结论:
- 更新的分类反映了在定义皮肤淋巴瘤和LPDs方面取得的重大进展.
- 临床,组织学,表型和分子特征的整合对于准确的诊断至关重要.
- 世界卫生组织第五次分类通过详细的标准提高了诊断特异性,并包括初级免疫缺陷中的反应性淋巴细胞增殖和LPD.
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