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具有ALK阳性的大B细胞淋巴瘤 (ALK + LBCL) 与异常的CD3表达
Jess Baker1,2, Sara L Zadeh1, Nadine S Aguilera3
1Department of Pathology, University of Virginia Health System, Charlottesville, VA, 22901-0214, USA.
Journal of hematopathology
|April 4, 2024
概括
无细胞淋巴瘤激酶阳性大B细胞淋巴瘤 (ALK+ LBCL) 是一种罕见的侵袭性癌症. 这一案例突出显示了一种不寻常的表现,部分CD3T细胞标志物表达,强调需要全面的诊断评估.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 免疫型定型 免疫型定型
背景情况:
- 无细胞淋巴瘤激酶阳性大B细胞淋巴瘤 (ALK+ LBCL) 是一种罕见的,攻击性的扩散大B细胞淋巴瘤亚型.
- 这种淋巴瘤通常对B细胞标记物 (CD20,CD19,CD22) 呈负性,对血细胞标记物 (CD138,CD38,MUM1) 呈阳性.
- 通常没有T细胞标志物表达,只有极少数例外.
研究的目的:
- 报告一个不寻常的ALK+LBCL病例在一个58岁的男性.
- 调查ALK+ LBCL中模两可的免疫表型所带来的诊断挑战.
- 探索这种淋巴瘤亚型中罕见的T细胞标志物表达的意义.
主要方法:
- 使用了流细胞计,免疫组织化学和光在位杂交 (FISH).
- 对IGH和TCR gamma进行了基因重组研究.
- 分析了特定细胞标记物和ALK基因破坏的组织部分.
主要成果:
- 瘤表达了CD45和血细胞标记物 (CD138,MUM1) 与卡帕限制,但缺乏典型的B细胞标记物 (CD20,CD79a,PAX5).
- 观察到强烈的细胞质ALK表达和FISH确认的ALK基因破坏.
- 检测到同时存在的IGH和TCR马基因重组,TCR克隆呈多克隆,这表明T细胞参与有限.
结论:
- ALK+ LBCL可以呈现含糊不清的免疫表型,需要广泛的抗体面板来准确诊断.
- 在这种情况下,部分CD3表达在ALK+LBCL中是罕见的,其临床意义需要进一步调查.
- 综合的诊断方法对于正确识别和表征罕见淋巴瘤亚型至关重要.
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