Introduction. T-cell acute lymphoblastic leukaemia (T-ALL) is an aggressive malignancy that is exceptionally difficult to cure after relapse. We have recently demonstrated that T-ALL expresses significantly higher levels of the enzyme aldo-keto reductase family 1 member C3 (AKR1C3) compared with B-cell ALL (B-ALL). To exploit these findings we developed an AKR1C3-activated prodrug, ACHM-025, as a potential targeted therapy for paediatric T-ALL.
Aims. (1) Determine the in vivo efficacy and AKR1C3 selectivity of ACHM-025 against a panel of T-ALL patient-derived xenografts (PDXs); (2) Compare the in vivo efficacy of ACHM-025 with paediatric ALL standard-of-care consolidation therapy.
Methods and Results. Standard in vivo drug evaluation uses large cohorts of mice per PDX (e.g. 6 control, 6 drug treated) against a small panel of PDXs. We tested ACHM-025 using a single mouse testing format (1 control, 1 drug treated), to enable a large number of PDXs to be tested. ACHM-025 (25 mg/kg, i.p. weekly × 3) was tested against 21 T-ALL PDXs. Remarkably, 7/21 PDXs treated with ACHM-025 never reached event, over 250 days after the last treatment. ACHM-025 efficacy showed a significant correlation with basal AKR1C3 protein expression (R2=0.34; p=0.0054). Both ACHM-025 and cyclophosphamide are DNA cross-linking prodrugs. Comparing single agents, ACHM-025 (5 mg/kg, i.p. weekly × 2) was significantly more effective than cyclophosphamide (75 mg/kg, i.p. weekly × 2) with a T-C of 25.3 versus 57.4 days (p=0.0005). Next, we compared the in vivo efficacy of standard-of-care consolidation therapy (Ara-C, 6-mercaptopurine and cyclophosphamide) with the combination of Ara-C, 6-mercaptopurine and ACHM-025 against a T-ALL PDX derived from a patient at relapse. The combination of Ara-C, 6-mercaptopurine and ACHM-025 was significantly more effective than standard-of-care consolidation therapy, more than doubling survival (T-C) from 36.3 to 74.7 days (p=0.0005).
Conclusion. This study provides strong preclinical evidence highlighting the potential of ACHM-025 as a targeted and effective therapy for aggressive forms of T-ALL, with AKR1C3 expression as a predictive biomarker of its activity.