急性淋巴细胞白血病与淋巴母细胞淋巴瘤是同一种疾病吗？

2020年Emma Kroeze, 通过基因测序表明两种疾病是同一种疾病的不同表现形式。该文献通过最新的检验技术检测以前的假设和推论。为急性淋巴细胞白血病方案在淋巴母细胞淋巴瘤中的应用提供了理论基础。

T细胞淋巴母细胞淋巴瘤(T- lbl)和淋巴母细胞白血病(T- all)是由未成熟前体细胞转化而产生的恶性肿瘤。T-LBL和T-ALL的相似之处提出了这些实体是代表一种疾病还是反映两种不同疾病的问题。在过去的20年里，对T-ALL的基因图谱已经进行了彻底的研究，而对T-LBL的基因驱动突变知之甚少。然而，T-LBL和T-ALL独立研究的临床、免疫表型和分子观察结果的比较，为T-LBL和T-ALL反映同一疾病不同表现的理论提供了支持。另外，T-LBL和T-ALL可能同时从一种常见的恶性前体细胞进化而来，每一种细胞都有其特定的致病需求或细胞依赖性，与单独的白血病细胞相比，淋巴组织中嵌入间质的母细胞之间存在差异。这篇综述的目的是将最近关于T-LBL和T-ALL患者的临床表现、遗传易感和可能导致基因表达特征差异的附加突变获取的研究结果集中在一起。对T-LBL与T-ALL关系的深入了解可能进一步有助于将经证实的T-ALL疗法应用于T-LBL患者。。

T-cell lymphoblastic lymphoma (T-LBL) and lymphoblastic leukemia (T-ALL) represent

malignancies that arise from the transformation of immature precursor T cells. Similarities

in T-LBL and T-ALL have raised the question whether these entities represent 1 disease or

reflect 2 different diseases. The genetic profiles of T-ALL have been thoroughly investigated

over the last 2 decades, whereas fairly little is known about genetic driver mutations in

T-LBL. Nevertheless, the comparison of clinical, immunophenotypic, and molecular

observations from independent T-LBL and T-ALL studies lent strength to the theory that

T-LBL and T-ALL reflect different presentations of the same disease. Alternatively, T-LBL

and T-ALL may simultaneously evolve from a common malignant precursor cell, each

having their own specific pathogenic requirements or cellular dependencies that differ

among stroma-embedded blasts in lymphoid tissues compared with solitary leukemia

cells. This review aims to cluster recent findings with regard to clinical presentation,

genetic predisposition, and the acquisition of additional mutations that may give rise to

differences in gene expression signatures among T-LBL and T-ALL patients. Improved

insight in T-LBL in relation to T-ALL may further help to apply confirmed T-ALL therapies

to T-LBL patients