Taken together, this is the first case of aggressive T-LGL leukemia with phenotype of TCRαβ and CD4-/CD8- which shows a CNS involvement during chemotherapy and responded well to T-PEPC metronomic regimen, suggesting that metronomic regimen should be a better treatment option for such patients. Here, CD8A is linked to T-cell large granular lymphocyte leukemia.