OBJECTIVETo evaluate the long-term survival of children and adolescents with lymphoblastic lymphoma (LBL) treated by a modified NHL-BFM-90 protocol.

METHODSFrom March 1998 to November 2010, 107 untreated patients with LBL (age <18 years) were enrolled and stratified into three groups (R1, R2 and R3), according to the stage of disease and response to induction chemotherapy. All patients received different intensive chemotherapy regimens based on a modified NHL-BFM-90 protocol. Total treatment duration was 2 years.

RESULTSOf the 107 patients, 79 were boys and 28 were girls, with a median age of 10 years (range 2.5-18 years). Six patients (5.6%) were stage I/II, 101 (94.4%) stage III/IV. The R1, R2 and R3 groups accounted for 5.6%, 71.0% and 23.4%, respectively. 75.7% of the patients had T-LBL, and 24.3% was B-LBL. At a median follow-up duration of 60 months (range 1-186 months), 24 patients died. The 5-year event-free survival (EFS) and overall survival (OS) were 75.5% and 77.8 % for all patients, 100.0% and 100.0% for group R1, 84.5% and 87.5 % for R2, 44.0% and 44.0% for R3, 72% and 73.5% for T-LBL, 86.4% and 88.5% for B-LBL, respectively. Myleosuppression was the major toxicity and need aggressive management.

CONCLUSIONThe modified NHL-BFM-90 protocol is an effective therapy for children and adolescents with LBL in low and intermediate risk. T-LBL had the similar outcomes as B-LBL did. The patients in high-risk group had a poor survival and new protocols are needed.