Clinical outcomes and Prognostic factors in children with
B-Lymphoblastic Lymphoma (pB-LBL) treated on Modified BFM-90 protocol:
experience from a tertiary cancer care center in India
Background: Pediatric B-Lymphoblastic lymphoma(pB-LBL) is a rare entity,
and appropriate treatment for pB-LBL is not well defined. While
intensive Acute Lymphoblastic leukemia(ALL) type regimens achieve long
term event free survival of 90% across western co-operative group
trials, published data from Asian studies on long term outcomes in
pB-LBL are scarce. We evaluated the outcomes and prognostic factors of
pediatric B-LBL patients treated at our center. Methods: We
retrospectively analyzed the data of pediatric B-LBL patients treated
between January 2010 and December 2017 on a uniform protocol(modified
BFM 90). Patients were evaluated for early response post-induction and
monitored for toxicity and long term outcomes. Kaplan-Meier method was
used to estimate the event free survival(EFS) and overall survival(OS).
Cox regression models were performed to identify prognostic factors.
Results: Of 21 patients who received treatment on the modified BFM 90
protocol, 17(81%) were alive in remission, 3(14%) had relapse, and
1(4%) had treatment-related mortality(TRM) while in remission. Two of 3
relapsed patients subsequently expired. With a median follow-up of 66
months(range 6–114), 5-year Event free survival(EFS) and overall
survival(OS) were 80%(95% CI:71–89%) and 91% (95% CI:85–97%),
respectively. While delayed presentation (≥3 months) had inferior
EFS(p-0.030), patients with elevated baseline Lactate Dehydrogenase(LDH)
had a worse OS(p-0.037). Age, gender, site of origin, stage, and
post-induction response had no bearing on outcome. Conclusions: Outcomes
of pB-LBL patients treated on modified BFM 90 protocol are excellent.
Higher disease burden manifested by elevated baseline LDH and delayed
presentation(≥3 months) portend poorer survival.