Department of Pathology, Medical College of Wisconsin, Milwaukee, WI 53226, USA.
Am J Clin Pathol. 2012 Mar;137(3):403-11. doi: 10.1309/AJCP7QHH5XASTJPL.
Flow cytometry (FC) is frequently used to detect aberrant peripheral blood (PB) T cells ("Sézary cells") in patients with mycosis fungoides (MF) and Sézary syndrome (SS). However, immunophenotypic stability of MF/SS over time is not well characterized. We analyzed 141 PB samples from 9 cases (2 SS, 7 MF). At diagnosis, there were 3 to 5 immunophenotypic aberrancies per case (median, 4), including dim or absent CD2, CD3, CD4, CD5, CD7, or CD26 and bright CD45RO. Of 9 patients, 7 had a subsequent change in immunophenotype. All patients retained multiple aberrancies at follow-up (median, 3 per analysis; range, 2-6), of which 22.0% (81/369) were new. In 5 patients, a more than 99% decrease in absolute Sézary cell (ASC) counts by FC after alemtuzumab therapy or total skin electron beam radiation was associated with clinical improvement. We observed minor immunophenotypic changes over time in most patients with MF/SS; however, the Sézary clones maintain persistently aberrant immunophenotypes and seem amenable to follow-up with limited FC panels. ASC counts by FC correlated well with clinical response.
流式细胞术 (FC) 常用于检测蕈样肉芽肿 (MF) 和 Sezary 综合征 (SS) 患者外周血 (PB) 中异常的 T 细胞 ("Sézary 细胞")。然而,MF/SS 随时间推移的免疫表型稳定性尚未得到很好的描述。我们分析了 9 例病例 (2 例 SS,7 例 MF) 的 141 份 PB 样本。在诊断时,每个病例有 3 到 5 种免疫表型异常 (中位数为 4),包括 CD2、CD3、CD4、CD5、CD7 或 CD26 表达减弱或缺失,以及 CD45RO 表达明亮。9 例患者中有 7 例随后发生免疫表型改变。所有患者在随访时仍保留多种异常 (中位数为每次分析 3 种;范围 2-6),其中 22.0% (81/369) 为新出现的异常。在 5 例患者中,阿仑单抗治疗或全身电子束照射后 FC 检测的绝对 Sézary 细胞 (ASC) 计数下降 99%以上与临床改善相关。我们观察到大多数 MF/SS 患者随时间出现轻微的免疫表型变化;然而,Sézary 克隆仍然保持持续异常的免疫表型,似乎可以通过有限的 FC 面板进行随访。FC 检测的 ASC 计数与临床反应相关性良好。