对41例血管免疫母细胞性T细胞淋巴瘤患者的皮肤活检结果进行回顾性分析,检测其滤泡辅助性T细胞(follicular helper T-cell,TFH)标记物及EB病毒(Epstein-Barr virus,EBV)的表达情况,并采用等位基因特异性聚合酶链反应的方法分析其RHOA(p.G17v)及IDH2(p.R172K/S)的突变情况。我们把所有病例分成4种形态学模式:(1)血管周围淋巴细胞低密度浸润(n=11), (2)血管周围异型淋巴组胞密集浸润,偶见炎细胞(n=13), (3)血管免疫母细胞性T细胞淋巴瘤样肿瘤细胞弥漫浸润(n=4), (4)其他模式(n=13)。观察到2例EBV+和2例浆细胞样淋巴组织增生性疾病。我们观察到TFH标记物在这些病例中的表达不同[CD10(50%)、BCLB6 (84%)、PD1(94%)、CXCL13(84%)和ICOS (97. 5%)],并观察到EBV+的B免疫母细胞(26%)。TFH表型在最具诊断挑战性的模式1和2中检出率分别为82%和73%。TFH标记物和EBV检测有助于诊断这些细胞低密度浸润病例。我们发现RHOA G17V和IDH2 R172K/S突变在皮肤病变中的检出率分别为14/18(78%)和3/16(19%)。在一部分具有诊断挑战性模式1和2的活检病例中可以检测到RHOA G17V突变。皮肤活检和淋巴结(lymph node, LN)活检均发现RHOA G17V突变的检出率为7/9(64%),只在皮肤或LN病变中发现RHOA G17V突变的各有1例。RHOA G17V突变在皮肤病变中的检出率与既往报到在LN病变中的检出率相似。RHOA G17V突变可能是血管免疫母细胞性T细胞淋巴瘤皮肤病变诊断的一个敏感标记物,尤其对诊断肿瘤细胞低密度浸润的病例有帮助。
Am J surg Pathol 2017;41;1581-1592
美国外科病理学杂志中文版2018年第二期摘要No.2
美国外科病理学杂志中文版2018年第二期摘要No.2
(王冠男 翻译 石怀银 审校)
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