Majcherek Maciej, Przeorski Krzysztof, Mroczkowska-Bękarciak Aleksandra, Nogaj Natalia, Szymczak Donata, Kopszak Anna, Kujawa Krzysztof, Jabłonowska-Babij Paula, Tomasiewicz Maciej, Szeremet Agnieszka, Wróbel Tomasz, Czyż Anna
Clinic of Hematology, Cellular Therapies and Internal Medicine, Wroclaw Medical University, 50-367 Wrocław, Poland.
Laboratory of Flow Cytometry and Cytomorphology, Department of Hematology and Bone Marrow Transplantation, University Hospital in Wroclaw, 50-556 Wroclaw, Poland.
Int J Mol Sci. 2025 Jul 30;26(15):7382. doi: 10.3390/ijms26157382.
Continuous development of molecular and immunophenotypic techniques enables more precise diagnoses and more accurate assessment of prognosis in myelodysplastic syndromes (MDS). However, the relationship between genetic alterations and immunophenotype remains very poorly understood. The analysis included 30 patients diagnosed at a tertiary center who were eligible for azacitidine treatment. Next-generation sequencing (NGS) was performed at the start of the study to assess the mutation status of 40 genes associated with MDS pathogenesis. In addition, multiparametric flow cytometry (MFC) was performed to assess the ELN score (Ogata score) and, additionally, to detect an abnormal CD11b/HLA-DR and CD11b/CD13 expression pattern. In the studied patient population, higher ELN score results were found in patients with mutations in epigenetic modifiers and pathogenic mutations of the tumor suppressor genes. Signal pathway mutations were associated with lower platelet counts at diagnosis. The results of this study indicate a correlation between molecular abnormalities and deviations in cell immunophenotype. Investigating this correlation may, in the future, allow the development of new scales that allow a more sensitive and specific diagnosis of MDS and a more precise prediction of its course.
分子和免疫表型技术的不断发展使得骨髓增生异常综合征(MDS)的诊断更加精确,预后评估更加准确。然而,基因改变与免疫表型之间的关系仍知之甚少。该分析纳入了30例在三级中心确诊且符合阿扎胞苷治疗条件的患者。在研究开始时进行了二代测序(NGS),以评估与MDS发病机制相关的40个基因的突变状态。此外,进行了多参数流式细胞术(MFC)以评估ELN评分(绪方评分),并额外检测异常的CD11b/HLA-DR和CD11b/CD13表达模式。在研究的患者群体中,表观遗传修饰因子突变和肿瘤抑制基因致病突变的患者ELN评分结果更高。信号通路突变与诊断时较低的血小板计数相关。本研究结果表明分子异常与细胞免疫表型偏差之间存在相关性。未来,对这种相关性的研究可能会促成新量表的开发,从而实现对MDS更敏感、更特异的诊断以及对其病程更精确的预测。