Ujj Zsófia, Buglyó Gergely, Udvardy Miklós, Vargha György, Biró Sándor, Rejtő László
Institute of Internal Medicine, Division of Haematology, University of Debrecen, Nagyerdei krt. 98, 4032, Debrecen, Hungary.
Pathol Oncol Res. 2014 Jul;20(3):565-70. doi: 10.1007/s12253-013-9729-7. Epub 2013 Dec 28.
The Wilms tumor 1 (WT1) gene has a complex role as a transcriptional regulator, acting as tumor suppressor or oncogene in different malignancies. The prognostic role of its overexpression has been well-studied in leukemias, especially acute myeloid leukemia (AML), but not in lymphomas. For the first time to our knowledge, we present a study demonstrating the correlation of WT1 expression and survival in various non-Hodgkin lymphomas. We also studied the prognostic implications of WT1 overexpression in adult acute lymphoblastic leukemia (ALL). In our sample of 53 patients--25 with diffuse large B-cell lymphoma (DLBCL), 8 with mantle cell lymphoma (MCL), 9 with peripheral T-cell lymphoma (PTCL), 2 with Burkitt's lymphoma, 2 with mucosa-associated lymphoid tissue (MALT) lymphoma, and 7 with B-cell ALL--, we measured WT1 mRNA from blood samples by quantitative RT-PCR, and divided the patients into subgroups based on the level of expression. Kaplan-Meier survival curves were drawn and compared using the logrank test. In the sample of DLBCL patients, the difference in overall and disease-free survival between WT1-positive and negative subgroups was significant (p = 0.0475 and p = 0.0004, respectively), and in a few observed cases, a sudden increase in WT1 expression signified a relapse soon followed by death. Disease-free survival curves in MCL and ALL were similarly suggestive of a potential role played by WT1. In PTCL, though WT1-positivity was detected in 4 out of 9 cases, it did not seem to affect survival. The few cases of MALT and Burkitt's lymphoma all proved to be WT1-negative.
威尔姆斯瘤1（WT1）基因作为转录调节因子具有复杂的作用，在不同的恶性肿瘤中既充当肿瘤抑制因子又充当癌基因。其过表达在白血病，尤其是急性髓系白血病（AML）中的预后作用已得到充分研究，但在淋巴瘤中尚未得到研究。据我们所知，我们首次进行了一项研究，证明WT1表达与各种非霍奇金淋巴瘤的生存率之间存在相关性。我们还研究了WT1过表达在成人急性淋巴细胞白血病（ALL）中的预后意义。在我们的53例患者样本中——25例弥漫性大B细胞淋巴瘤（DLBCL）、8例套细胞淋巴瘤（MCL）、9例外周T细胞淋巴瘤（PTCL）、2例伯基特淋巴瘤、2例黏膜相关淋巴组织（MALT）淋巴瘤和7例B细胞ALL——我们通过定量逆转录聚合酶链反应（RT-PCR）从血液样本中测量WT1 mRNA，并根据表达水平将患者分为亚组。绘制了Kaplan-Meier生存曲线，并使用对数秩检验进行比较。在DLBCL患者样本中，WT1阳性和阴性亚组之间的总生存期和无病生存期差异显著（分别为p = 0.0475和p = 0.0004），在一些观察到的病例中，WT1表达的突然增加预示着很快复发并随后死亡。MCL和ALL的无病生存曲线同样表明WT1可能发挥作用。在PTCL中，虽然9例中有4例检测到WT1阳性，但似乎并不影响生存。少数MALT和伯基特淋巴瘤病例均被证明为WT1阴性。
