Outcome and pathological classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy.

Nov 2012

Gerrard M, Waxman IM, Sposto R, Auperin A, Perkins SL, Goldman S, Harrison L, Pinkerton R, McCarthy K, Raphael M,Patte C, Cairo MS.

Source

Sheffield Children's Hospital, Sheffield, United Kingdom;

Abstract

Mediastinal large B-cell lymphoma (MLBL) represents only 2% of mature B-cell non-Hodgkin lymphoma (B-NHL) in patients ≤18 years of age. Gene expression profiling demonstrates that MLBL in adults more closely resembles classical Hodgkinlymphoma than it does diffuse large B-cell lymphoma (DLBCL). We analyzed data from childhood and adolescent patients with Stage III MLBL (N=42) and non MLBL DLBCL (N=69) treated with Group B therapy on the FAB/LMB 96 study. Demographics of MLBL patients: M/F: 26/16; median age 15.7 yrs (12.5-19.7); LDH <2 vs. ≥2 ULN: 23:19. Six MLBL patients (14%) had <20% response to initial COP-therapy. Central pathology classification revealed approximately 50% with classical features of primary mediastinal B-cell lymphoma (PMBL). Five-year event-free survival (EFS) for Stage III MLBL and non-MLBL DLBCL groups were 66% (95% CI: 49-78%) and 85% (95% CI: 71-92%), respectively, p<0.001 (14%). The 5-year overall survival (OS) in the 42 MLBL patients was 73% (95% CI: 56-84%). MLBL in adolescent patients is associated with significantly inferior EFS compared with stage III non-MLBL DLBCL and can be of multiple histologies. Alternate treatment strategies should be investigated in the future taking into account both adult MLBL approaches and more recent biological findings in adult MLBL.

Blood Journal