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The objective response rate was 9% (95% CI 0-41%). The clinical benefit rate was 73% (95% CI 39-94%). Four patients had grade 3 adverse events related to pembrolizumab. No patients experienced grade 4 or 5 adverse events or a catecholamine crisis. Progression free survival time was 5.7 months (95% CI 4.37-not reached). The median survival duration was 19 months (95% CI 9.9-not reached). PDL-1 expression and the presence of infiltrating mononuclear inflammatory cells in the primary tumor did not seem to be associated with disease respons