Superior vena cava syndrome and poor performance status at presentation affect survival in mediastinal T-lymphoblastic lymphoma—a single institute experience from India

Abstract

Analysis of mediastinal T-lymphoblastic lymphomas (T-LBL) with T-acute lymphoblastic leukemia has precluded identification of prognostic factors. Newly diagnosed T-LBL patients presenting with mediastinal mass and <20% bone marrow involvement were analyzed for clinical features and outcome. In 55 patients with median age 18 years, 39 (71%) had "B" symptoms, 46 (83.6%) had bulky mediastinal mass and two thirds of patients (n = 36) had superior vena cava syndrome/superior mediastinal syndrome (SVCS/SMS). With acute lymphoblastic leukemia (ALL)-like protocols in majority, complete remission was achieved in 58.2% patients. Estimated 5-year overall survival (OS) and event-free survival (EFS) were 59.8 and 51.6% (median follow-up-35 months). On multivariate analysis, poor Eastern Cooperative Oncology Group (ECOG) performance status (PS > 2; n = 14) affected OS (p = 0.007), while Poor ECOG PS and SVCS/SMS affected EFS (p = 0.008 and p = 0.035, respectively). Combination of baseline-poor PS and presence of SVCS/SMS predicted poor EFS in a prognostic model (HR 6.20; p = 0.002). This is the first outcome study from Asia which has been able to predict baseline ECOG PS and presence of SVCS/SMS as prognostic factors affecting EFS.