The Eµ-Myc transgenic mouse provides a tractable tool for dissecting mechanisms of B-lineage lymphomagenesis and efficacy of novel therapeutics. We performed genomic profiling of Eµ-Myc lymphomas to identify genetic lesions co-operating with Myc in de novo lymphomagenesis. Whole genome sequencing and FISH mapped the original insertion of the Eµ-Myc transgene into chromosome 19 with associated germline amplification of an adjacent chromosomal segment including the loci for Jak2 and Cd274 (encoding PD-L1). Additional copy number gains of this amplicon occurred in half of sequenced tumours indicating potential co-operativity between the Myc transgene and adjacent amplified elements such as Jak2. Importantly, amplification of the syntenic human chromosome 9p24.1 is described in primary mediastinal B-cell lymphoma and Hodgkin lymphoma putatively conferring increased signalling through the canonical JAK/STAT pathway and increased PD-L1 transcript abundance.1 We therefore hypothesised this amplicon to be functionally oncogenic to Eµ-Myc lymphoma.
Eµ-Myc lymphomas did not show biochemical evidence of activated canonical JAK/STAT signalling despite Jak2 copy-number gains. Moreover, Eµ-Myc lymphomas were resistant to pharmacological JAK2 inhibition in vitro and in vivo. Over-expression of constitutively active (V617F) JAK2 in Eµ-Myc haematopoietic progenitor cells failed to accelerate lymphomagenesis as compared to nRAS Q61K-controls. Consistent with the JAK2 data, surface expression of PD-L1 by FACS analysis did not correlate with amplicon dose and, in contrast to a PD-L1HI MC38 colon-cancer cell line, JAK2 inhibition did not down-regulate PD-L1 expression on Eµ-Myc cells. However, in vivo treatment with PD-L1 blocking antibody improved survival in lymphoma-bearing mice.
These results indicate that Jak2 does not co-operate with Myc in lymphomagenesis and that pharmacological inhibition of JAK2 is not a favourable therapeutic strategy in aggressive Myc-driven lymphoma. Interestingly however, PD-L1 expression levels were not predictive of in vivo responses suggesting that the efficacy of PD-L1 blockade is not restricted to tumours with genetic amplification. The functional significance of 9p amplifications in human lymphoma requires further investigation.