Background: Killer-cell Ig-like receptors expressed on natural killer (NK) cells and their ligands on target cells may predict outcome in patients (pts) with high-risk relapsed/refractory neuroblastoma (NB) treated by LTI of ch14.18/CHO.
Methods: 53 pts received 6x106 IU/m2 sc IL-2 (d1-5; 8-12), LTI of 100 mg/m2 ch14.18/CHO (d8-17) and 160 mg/m2 oral 13-cis-RA (d19-32) in a closed single center program (53 pts) (APN311-303). Polymorphisms in Fcγ-receptor genes 2A (H131R), -3A (V158F) and -3B (NA1/NA2), and KIR- as well as KIR ligand expression (HLA-C1; HLA-C2; HLA-Bw4+) were determined by real-time PCR.
Results: A best response rate of 40.5% translated into a 5-y OS of 56.4±7.1% (mean OS 4.35y [0.3-6.2y]) and a 5-y PFS of 29.1±6.3% (mean PFS 2.4y [0.1 - 5.9y]). Median TTP/PFS was 1.35 y (95% CI: 0.21-2.48 y). This result is clearly superior to historical controls not treated with ch14.18/CHO with a 5-y OS of 14.8±6.8% (p<0.005), indicating clinical efficacy of the treatment.
We identified 21/53 pts with the NK- stimulatory KIR haplotype (2DS2+) who had superior OS- and PFS-rates compared to 16/53 pts with an inhibitory haplotype and KIR/KIR-ligand match (2DS2-, 3DL1+, Bw4+) (p<0.02). Similarly, we found 33/53 pts with low affinity FCGR alleles (FCGR2A-H131R/R and/or FCGRA3A-V158 F/F). These patients showed lower PFS rates compared to 19/53 patients with high affinity FCGR polymorphisms (p<0.01).
Importantly, ADCC analysis on day 15 of cycle 1 showed higher levels in the pt group with NK- stimulatory versus NK- inhibitory KIR/KIR ligand haplotypes (27±5 % vs. 12±3%, p< 0.01). A similar effect was observed in pts with high affinity FCGR polymorphisms with an ADCC increase of 21±7% compared to 11±2% in the low affinity FCGR control.
Conclusion: KIR-haplotypes and FCGR- polymorphisms correlated with the functional immune parameter ADCC and clinical outcome, and may therefore be useful biomarkers for LTI with ch14.18/CHO.