Clin Lymphoma Myeloma Leuk 2019 06 8;19(6):e291-e298. Epub 2019 Feb 8.
Department of Medical Oncology, Dr BRA IRCH, All India Institute of Medical Sciences, New Delhi, India. Electronic address:
Background: There is a lack of clinical predictors for prognosticating lymphoblastic lymphoma (LBL). In view of this lacuna, we evaluated outcomes and prognostic factors for LBL treated with a uniform protocol at our center.
Patients And Methods: This study included consecutive patients of pediatric LBL aged ≤18 years from January 2003 to January 2017. Patients were staged using the St Jude staging system. All patients were treated with acute lymphoblastic leukemia like BFM90 protocol. The Kaplan-Meier method was used for survival analysis. A statistical model was made using stepwise regression and forward selection of the factors predicting event-free survival (EFS) and overall survival (OS).
Results: Sixty-five patients were evaluated with a median age of 12 years (range, 1-18 years) and male:female ratio of 2.25:1. Fifty-four patients presented with mediastinal disease. Median follow-up was 54.57 months (range, 0.6-140.5 months). EFS at 10 years was 62 ± 6% (95% confidence interval [CI], 0.49-0.73) and OS 71 ± 5% (95% CI, 0.57-0.81). In multivariate analysis, symptom duration ≤30 days, white blood cell (WBC) count >12000/µL and serum albumin ≤3.5 g/dL predicted inferior EFS and OS. A prognostic model with these 3 factors suggested that those without any of these risk factors had an OS of 92 ± 5% whereas those with 2 or 3 factors had an OS of 37 ± 14%.
Conclusion: Our outcomes are 15% to 20% lower than in the published literature. Low albumin level, high WBC count at baseline, and symptom duration <30 days emerged as adverse predictors for EFS and OS. These clinical predictors and prognostic model for pediatric LBL should be validated in prospective cohorts.