Giudice Valentina, Gorrese Marisa, Vitolo Rosa, Bertolini Angela, Marcucci Rossella, Serio Bianca, Guariglia Roberto, Ferrara Idalucia, Pepe Rita, D'Alto Francesca, Izzo Barbara, Pedicini Antonio, Montuori Nunzia, Langella Maddalena, Selleri Carmine
Department of Medicine, Surgery and Dentistry, "Scuola Medica Salernitana", University of Salerno, 84081 Baronissi, Salerno, Italy.
Hematology and Transplant Center, University "Hospital San Giovanni di Dio e Ruggi D'Aragona", 84131 Salerno, Italy.
Biomedicines. 2021 Apr 6;9(4):387. doi: 10.3390/biomedicines9040387.
Wilm's tumor 1 (), a zinc-finger transcription factor and an epigenetic modifier, is frequently overexpressed in several hematologic disorders and solid tumors, and it has been proposed as diagnostic and prognostic marker of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). However, the exact role of in leukemogenesis and disease progression remains unclear. In this real-world evidence retrospective study, we investigated prognostic role of WT1-mRNA expression levels in AML and MDS patients and correlations with complete blood counts, flow cytometry counts, and molecular features. A total of 71 patients (AML, = 46; and MDS, = 25) were included in this study, and levels were assessed at diagnosis, during treatment and follow-up. We showed that expression levels were inversely correlated with normal hemopoiesis in both AML and MDS, and positively associated with blast counts. Flow cytometry was more sensitive and specific in distinguishing normal myeloid cells from neoplastic counterpart even just using linear parameters and CD45 expression. Moreover, we showed that a simple integrated approach combining blast counts by flow cytometry, mutational status, and expression levels might be a useful tool for a better prognostic definition in both AML and MDS patients.
肾母细胞瘤1(WT1)是一种锌指转录因子和表观遗传修饰因子,在多种血液系统疾病和实体瘤中经常过度表达,并且已被提议作为急性髓系白血病(AML)和骨髓增生异常综合征(MDS)的诊断和预后标志物。然而,WT1在白血病发生和疾病进展中的确切作用仍不清楚。在这项真实世界证据回顾性研究中,我们调查了WT1 - mRNA表达水平在AML和MDS患者中的预后作用以及与全血细胞计数、流式细胞术计数和分子特征的相关性。本研究共纳入71例患者(AML,n = 46;MDS,n = 25),并在诊断时、治疗期间和随访期间评估WT1水平。我们发现,WT1表达水平在AML和MDS中均与正常造血呈负相关,与原始细胞计数呈正相关。即使仅使用线性参数和CD45表达,流式细胞术在区分正常髓系细胞与肿瘤对应细胞方面也更敏感和特异。此外,我们表明,一种简单的综合方法,结合流式细胞术的原始细胞计数、WT1突变状态和WT1表达水平,可能是更好地定义AML和MDS患者预后的有用工具。