Diffuse Midline Glioma (otherwise known as DMG or DIPG, Diffuse Intrinsic Pontine Glioma) is a rare but devastating brain tumour of childhood: median age of diagnosis is 5 – 9 years of age, with 2 year overall survival of <10%.1 The only proven treatment is palliative radiotherapy that extends survival but does not provide cure.1 Recurrent mutations in Histone 3.3, resulting in a replacement of lysine by methionine at position 27 (K27M), are one of the most common, key driver mutations in DMG tumourigenesis.2 Patients who are HLA-A2 positive present a novel peptide at the cell surface derived from this K27M mutation;3 this HLA-A2 restricted H3K27M epitope has been the target of immune vaccine and T cell receptor-based approaches.4, 5
Chimeric Antigen Receptor(CAR)-T cell therapy is a promising form of adoptive cell therapy, that re-engineers patient-derived T cells to express a hybrid receptor specific to a tumour-specific antigen of choice.6 CAR-T therapy directed against the CD-19 antigen has been extraordinarily successful in various haematological malignancies,7 and its tumour-specific nature makes this therapy very appealing to apply to DMG.
We generated single chain variable fragments (scFv) against the novel H3K27M- HLA-A2 peptide-MHC complex by Retained Display screening;8 Clone 1 and Clone 2. These were cloned into a second generation CAR construct, and demonstrated cytotoxic function against H3K27M-HLA-A2 positive T2 cells in vitro, as well as corresponding cytokine release data.