Frazer JK, Li KJ, Galardy PJ, Perkins SL, Auperin A, Anderson JR, Pinkerton R, Buxton A, Gross TG, Michon J, Leverger G, Weinstein HJ, Harrison L, Shiramizu B, Barth MJ, Goldman SC, Patte C, Cairo MS. Excellent outcomes in children and adolescents with CNS+ Burkitt lymphoma or other mature B-NHL using only intrathecal and systemic chemoimmunotherapy: results from FAB/LMB96 and COG ANHL01P1. Br J Haematol. 2019 Apr;185(2):374-377. doi: 10.1111/bjh.15520. Epub 2018 Aug 16. PMID: 30117142; PMCID: PMC6588172.
Study ID Citation
Abstract
Burkitt lymphoma (BL) is the most common Non-Hodgkin lymphoma (NHL) in children, representing 40–50% of paediatric NHL (Cairo et al, 2012). BL is frequently advanced, involving the bone marrow (BM), central nervous system (CNS) or both, which requires aggressive therapy. BL is also the most frequently CNS-positive (CNS+) paediatric NHL (9–13% of cases) (Cairo et al, 2007, 2012; Patte et al, 2007; Gerrard et al, 2008). Trials have strived to improve outcomes in CNS+ BL and other mature B cell NHL (B-NHL), where prognoses are inferior (Salzburg et al, 2007). Specifically, CNS+ patients who were also BM-positive (BM+) had the worst outcomes on the French-American-British (FAB)/Lymphome Malins B (LMB)96 trial (Cairo et al, 2012). Yet most patients do achieve long-term survival, so studies also aim to reduce therapy-induced sequelae. The international FAB/LMB96 cooperative group trial previously demonstrated that CNS+ B-NHL patients treated with intensified CNS-directed systemic and intrathecal (IT) therapies had similar event-free and overall survival (EFS, OS) to prior CNS radiotherapy-containing regimens (Cairo et al, 2007). Besides altering CNS-directed treatment, FAB/LMB96 also randomized standard-versus reduced-intensity arms: reduced-intensity was proven inferior (72% vs. 84% EFS) (Cairo et al, 2007). Here, we focus CNS+ patients treated on the standard FAB/LMB96 C1-arm.