Oral Presentation 33rd Lorne Cancer Conference 2021

T-ALL can evolve to oncogene independence (#36)

Hesham Abdulla 1 , Anh Vo 2 , Benjamin Shields 2 , Tenae Davies 2 , Jacob Jackson 2 , Raed Alserihi 1 3 , Elizabeth M Viney 1 , Tin Yang Wong 2 , Feng Yan 2 , Nicholas Wong 2 , Lisa Demoen 4 , David Curtis 2 , Warren Alexander 1 , Pieter Van Vlierberghe 4 , Ross Dickins 2 , Matthew P McCormack 2
  1. The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, Australia
  2. Australian Centre for Blood Diseases, Monash University, Melbourne, VIC, Australia
  3. King Abdul-Aziz University, Jeddah, Saudi Arabia
  4. Cancer Research Institute Ghent (CRIG), Ghent, Belgium

The majority of cases of T-cell acute lymphoblastic leukemia (T-ALL) contain chromosomal abnormalities that drive overexpression of oncogenic transcription factors. However, whether these initiating oncogenes are required for leukemia maintenance is poorly understood. To address this, we developed a tetracycline-regulated mouse model of T-ALL driven by the oncogenic transcription factor Lmo2. This revealed that whilst thymus-resident pre-Leukemic Stem Cells (pre-LSCs) required continuous Lmo2 expression, the majority of leukemias relapsed despite Lmo2 withdrawal. Relapse was associated with a mature phenotype and frequent mutation or loss of tumor suppressor genes including Ikzf1 (Ikaros), with targeted deletion Ikzf1 being sufficient to transform Lmo2-dependent leukemias to Lmo2-independence. Moreover, we found that the related transcription factor TAL1 was dispensable in several human T-ALL cell lines that contain SIL-TAL1chromosomal deletions driving its overexpression, indicating that evolution to oncogene independence can also occur in human T-ALL. Together these results indicate an evolution of oncogene addiction in murine and human T-ALL and show that loss of Ikaros is a mechanism that can promote self-renewal of T-ALL lymphoblasts in the absence of an initiating oncogenic transcription factor.