A case of rare pediatric unclassified NK/T cell lymphoma

Abstract

The article presents a case report of rare unclassified peripheral T-cell lymphoma (PTCL) in a child. The disease was characterized by refractory course and addition of immune checkpoint inhibitors (ICI) to chemotherapy resulted in remission induction. The rationale for im-munotherapy was presence of programmed cell death ligand 1 (PDL1) on the cells of tumor microenviron-ment. First remission was consolidated with autologous hematopoietic stem cell transplantation (HSCT), and second remission was followed by allogeneic HSCT. Post-transplant period was complicated by severe acute graft-versus-host disease (GVHD) successfully man-aged with ruxolitinib. To perform local control, intrath-ecal cytostatic therapy was used prior to HSCT and post-transplant. Three years after last transplantation, the patient is disease-free and GVHD-free, with overall good quality of life and only mild impairment of cogni-tive functions.