Rampal Raajit, Figueroa Maria E
Leukemia Service, Memorial Sloan Kettering Cancer Center, New York, NY, USA
Department of Pathology, University of Michigan Medical School, Ann Arbor, MI, USA
Haematologica. 2016 Jun;101(6):672-9. doi: 10.3324/haematol.2015.141796.
Wilms tumor 1 (WT1) has long been implicated in acute myeloid leukemia. It has been described to be both overexpressed and mutated in different forms of acute myeloid leukemia, and overexpression has been reported to play a prognostic role in this disease. However, the precise mechanism through which WT1 may play a role in leukemogenesis has remained elusive. In recent years, new evidence has emerged that points towards a novel role of WT1 mutations in the deregulation of epigenetic programs in leukemic cells through its interaction with TET proteins. Herein we review the current status of the field and its therapeutic and prognostic implications in acute myeloid leukemia.
肾母细胞瘤1(WT1)长期以来一直被认为与急性髓系白血病有关。在不同类型的急性髓系白血病中,WT1被描述为既过度表达又发生突变,并且据报道其过度表达在这种疾病中具有预后作用。然而,WT1在白血病发生过程中发挥作用的确切机制仍不清楚。近年来,新的证据表明,WT1突变通过与TET蛋白相互作用,在白血病细胞表观遗传程序失调中发挥新的作用。在此,我们综述了该领域的现状及其在急性髓系白血病中的治疗和预后意义。