成人t细胞淋巴母细胞淋巴瘤的遗传特征、治疗方式和预后模式的综合观点。

IF 1.5 Q3 HEMATOLOGY
Qihua Zou, Shuyun Ma, Xiaopeng Tian, Qingqing Cai
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引用次数: 1

摘要

成人t细胞淋巴母细胞淋巴瘤(T-LBL)是一种罕见的侵袭性非霍奇金淋巴瘤亚型,与儿童T-LBL不同,预后较差。由于其罕见性,对成人T-LBL的遗传和分子特征、最佳治疗方式和预后因素知之甚少。因此,我们对已有的研究进行总结,对上述问题进行全面探讨。NOTCH1/FBXW7、PTEN、RAS和KMT2D基因突变,以及JAK-STAT信号通路异常激活。我们还讨论了治疗方式。一旦确诊,成人T-LBL患者应尽快接受强化治疗或儿童急性淋巴细胞白血病治疗方案和中枢神经系统预防,并适当采用颅脑无辐射治疗方案。纵隔放射治疗改善了临床结果,但不良事件令人担忧。有高危因素的成人T-LBL患者或复发/难治性疾病患者可考虑进行造血干细胞移植。此外,基于5- mirnas的分类器、基于11-基因的分类器、基于4- cpg的分类器等新型预后模型的构建,对成人T-LBL具有重要的预后价值。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Comprehensive view on genetic features, therapeutic modalities and prognostic models in adult T-cell lymphoblastic lymphoma.

Adult T-cell lymphoblastic lymphoma (T-LBL) is a rare and aggressive subtype of non-Hodgkin's lymphoma that differs from pediatric T-LBL and has a worse prognosis. Due to its rarity, little is known about the genetic and molecular characteristics, optimal treatment modalities, and prognostic factors of adult T-LBL. Therefore, we summarized the existing studies to comprehensively discuss the above issues in this review. Genetic mutations of NOTCH1/FBXW7, PTEN, RAS, and KMT2D, together with abnormal activation of signaling pathways, such as the JAK-STAT signaling pathway were described. We also discussed the therapeutic modalities. Once diagnosed, adult T-LBL patients should receive intensive or pediatric acute lymphoblastic leukemia regimen and central nervous system prophylaxis as soon as possible, and cranial radiation-free protocols are appropriate. Mediastinal radiotherapy improves clinical outcomes, but adverse events are of concern. Hematopoietic stem cell transplantation may be considered for adult T-LBL patients with high-risk factors or those with relapsed/refractory disease. Besides, several novel prognostic models have been constructed, such as the 5-miRNAs-based classifier, 11-gene-based classifier, and 4-CpG-based classifier, which have presented significant prognostic value in adult T-LBL.

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CiteScore
1.70
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