Treatment of non-Hodgkin lymphoma (NHL) is an example of successful therapy of cancer in children with a cure rate approximating 80%. Unfortunately, relapsed NHL has a dismal outcome, and customary treatment is by highly toxic chemotherapy followed by hematopoietic stem cell transplantation (HSCT).
To analyze prognostic factors, and to report treatment outcome of relapsing/refractory mature B cell NHL.
A retrospective analysis including all patients less than 18 years initially diagnosed as mature B cell NHL who were primary refractory to chemotherapy or relapsed during the period between July 2012 till end of 2017 at the Children Cancer Hospital Egypt (CCHE).
Out of 494 patients diagnosed during the study period, 34 (7%) were included in our study. Twenty-three (67.6%) had Burrkitt lymphoma, while 5 (14.7%) had diffuse large B cell lymphoma. The majority were males (79.4%), with a median age 6.2 years. According to Modified Murphy Staging, 6 patients (17.6%) were stage II, 24 (70.6%) stage III, and 4 (11.8%) stage IV. Bone marrow (BM) involvement at presentation was detected in 2 patient, and 3 (8.8%) had CNS infiltration. Thirty patients (88.2%) received LMB protocol, and 4 (11.8%) R-CHOP as primary chemotherapy. One patient (2.9%) was treated as low risk, 28 (82.4%) as intermediate, and 5 (14.7%) high risk. Median delay in 1st line treatment was 22 days (range 0 to 162).Twenty two patients (64.7%) relapsed, and 12 (35.3%) had tumor progression. Relapse was early in 15 (68.2%), and late in 7 patients (31.8%). It was documented by tissue biopsy in 30 patients (88.2%), and bone marrow aspirate in 3 (8.8%). Patients relapsed other than primary site were 6/34 (17.6%). CNS relapse was detected in 8 (23.5%), BM in 8 (20.5%), while combined BM and CNS in 2 (5.9%). R-ICE was the 2nd line of treatment in 29 patients (85.3%), and complete 2nd remission (CR) was achieved in 7 patients (20.6%). Allogeneic HSCT was done for 2 patients (5.9%), and autologous HSCT for 3 patients (8.8%). Finally, 7/34 (20.5%) patients were alive in CR, and 24/37 (70.5%) died. The 3 years Overall survival (OS) 35.3%, with no statistical difference between relapsing and progression. The 3 years OS for patient who underwent HSCT was 80% compared 20% for no HSCT (p = 0.034).
Our relapse rate is higher than the literature probably due to delay of chemotherapy with loss of dose intensity. For patients in second remission, HSCT markedly improves the outcome.