2020 Volume 57 Issue 2 Pages 157-161
A 6-year-old boy presented with cough and dyspnea. Computed tomography revealed a mediastinal mass and left-side plural effusion. T-lymphoblastic lymphoma was diagnosed on the basis of cytologic findings of the pleural fluid. Following induction therapy, radiological examination confirmed complete remission. However, after five chemotherapy courses, positron emission tomography showed abnormally high uptake in the anterior mediastinum. The mass was biopsied; examination revealed a relapse and a t(9;17)(q34;q23) chromosome abnormality. He showed poor response to the ifosfamide, carboplatin, and etoposide (ICE) regimen and nelarabine, and bone marrow involvement was observed following the chemotherapy. He underwent cord blood transplantation (CBT), with a myeloablative regimen comprising 180 mg/m2 melphalan and 12 Gy of total body irradiation at nonremission status; however, a bone marrow relapse was detected on day 21. He died of disease progression on day 70 after CBT. Considering the adverse prognostic features related to this chromosomal abnormality, identification of the fusion gene and novel strategies, including molecular targeting therapy, are warranted.