Singh Jay, Kumar Rajive, Verma Deepak, Rajput Nishi, Palanichamy Jayanth Kumar, Sharma Gunjan, Bakhshi Sameer, Sharma Atul, Pushpam Deepam, Seth Rachna, Ranjan Amar, Tanwar Pranay, Singh Archna, Arora Mohit, Kumari Sarita, Chopra Anita
Laboratory Oncology Unit, Dr. BRAIRCH, AIIMS New Delhi, India.
Mahavir Cancer Sansthan Patna, Bihar, India.
Am J Blood Res. 2020 Oct 15;10(5):294-304. eCollection 2020.
Emerging evidence suggests existence of three prognostically relevant molecular entities among immature T-ALL-early thymic precursor ALL (ETP-ALL), T-ALL with the absence of biallelic deletion of TCRγ chains (ABD) and (Myocyte Enhancer Factor 2C) high T-ALL. However, the usefulness of ETP-ALL immunophenotype and assessment of ABD for this purpose has been questioned and, has not been studied in much detail. In this prospective analysis of 143 T-ALL patients, we evaluated the mutual association of these three entities and also determined how immunophenotypically-defined poor prognosis immature T-ALL relates to these entities. We found that all three of them, especially ABD, nearly completely characterized the immature group. High expression reflected ETP-ALL somewhat poorly and a few ABD and -high patients had non-immature immunophenotype-findings, that though in accord with published literature, call for exploration per T-cell receptor (TCR) classification scheme. ETP-ALL and high but not ABD had a higher frequency of minimal residual disease positivity and poor event-free survival. high, not ETP-ALL immunophenotype or ABD, had poorer overall survival. The value of ETP-ALL immunophenotype and status, as indicators of poor treatment response, needs further evaluation for possible incorporation in standard T-ALL management practice.
新出现的证据表明,在未成熟T细胞急性淋巴细胞白血病-早期胸腺前体急性淋巴细胞白血病(ETP-ALL)、无TCRγ链双等位基因缺失(ABD)的T细胞急性淋巴细胞白血病以及肌细胞增强因子2C(MEF2C)高表达的T细胞急性淋巴细胞白血病中存在三种与预后相关的分子实体。然而,ETP-ALL免疫表型及为此目的对ABD进行评估的实用性受到了质疑,且尚未进行详细研究。在对143例T细胞急性淋巴细胞白血病患者的这项前瞻性分析中,我们评估了这三种实体之间的相互关联,并确定了免疫表型定义的预后不良未成熟T细胞急性淋巴细胞白血病与这些实体的关系。我们发现,这三者,尤其是ABD,几乎完全可以界定未成熟组。MEF2C高表达对ETP-ALL的反映有些欠佳,少数ABD及MEF2C高表达患者具有非未成熟免疫表型结果,尽管这与已发表的文献一致,但仍需要根据T细胞受体(TCR)分类方案进行探索。ETP-ALL和MEF2C高表达而非ABD具有更高的微小残留病阳性率及较差的无事件生存率。MEF2C高表达而非ETP-ALL免疫表型或ABD具有更差的总生存率。ETP-ALL免疫表型及MEF2C状态作为治疗反应不佳指标的价值,需要进一步评估,以便可能纳入标准的T细胞急性淋巴细胞白血病管理实践中。