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流式细胞术在骨髓增生异常综合征诊断和预后分层中的可靠性:与形态学和突变谱的相关性。

Reliability of flow-cytometry in diagnosis and prognostic stratification of myelodysplastic syndromes: correlations with morphology and mutational profile.

机构信息

Department of Biomedicine and Prevention, Tor Vergata University, Rome, Italy.

UniCamillus-Saint Camillus International University of Health Sciences, Rome, Italy.

出版信息

Ann Hematol. 2023 Nov;102(11):3015-3023. doi: 10.1007/s00277-023-05384-2. Epub 2023 Aug 3.

Abstract

Diagnosis and prognostic stratification of myelodysplastic syndromes (MDS) have been complemented by new techniques, including flow cytometry and NGS. To analyze the relationship between molecular and cytofluorimetric data, we enrolled in this retrospective study, 145 patients, including 106 diagnosed with MDS and 39 controls. At disease onset, immunophenotypic (IF), cytogenetic tests, and cytomorphological (CM) examination on bone marrow were carried out in all patients, while NGS was performed in 58 cases. Ogata score presented a specificity of 100% and a sensitivity of 59%. The detection of at least two phenotypic aberrancies in Ogata negative patients increased the sensitivity to 83% and specificity to 87%. Correlations were identified between IF aberrancies and mutations, including positive Ogata>2 and mutations in SRSF2 (p=0.035), CD15 and U2AF1 (0.032), CD56 and DNMT3A (p=0.042), and CD38 and TP53 (p=0.026). In multivariate analysis, U2AF1 mutations, associated with del(20q) and/or abnormalities of chromosome 7 (group 4 as defined by the EuroMDS score), significantly correlated with an inferior overall survival (p=0.019). These parameters and Ogata score>2 also showed a significant correlation with inferior event-free survival (p=0.023 and p=0.041, respectively). Both CM and FC features correlated with prognosis and mutational patterns. In an integrated MDS work-up, these tools may guide indications for mutational screening for optimal risk stratification.

摘要

骨髓增生异常综合征(MDS)的诊断和预后分层已通过新的技术得到补充,包括流式细胞术和 NGS。为了分析分子和细胞荧光数据之间的关系,我们对 145 例患者进行了回顾性研究,其中包括 106 例 MDS 患者和 39 例对照组。在疾病发作时,所有患者均进行免疫表型(IF)、细胞遗传学检测和骨髓细胞形态学(CM)检查,而 58 例患者进行了 NGS。Ogata 评分的特异性为 100%,敏感性为 59%。在 Ogata 阴性患者中检测到至少两种表型异常可将敏感性提高到 83%,特异性提高到 87%。IF 异常与突变之间存在相关性,包括阳性 Ogata>2 和 SRSF2 突变(p=0.035)、CD15 和 U2AF1 突变(0.032)、CD56 和 DNMT3A 突变(p=0.042)和 CD38 和 TP53 突变(p=0.026)。在多变量分析中,U2AF1 突变与 del(20q)和/或染色体 7 异常(EuroMDS 评分定义的 4 组)相关,与总体生存较差显著相关(p=0.019)。这些参数和 Ogata 评分>2 也与无事件生存显著相关(p=0.023 和 p=0.041)。CM 和 FC 特征均与预后和突变模式相关。在综合 MDS 检查中,这些工具可能指导突变筛查的适应证,以实现最佳风险分层。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/55ad/10567902/d07ecc7fed4e/277_2023_5384_Fig1_HTML.jpg

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