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.

Shyam Srinivasan

and 10 more

Background: Even though rituximab has emerged as the standard of care for management of high risk paediatric burkitt lymphoma(BL) its safety in children from the low-middle income countries(LMICs) remains to be proven. We herein report our experience of using rituximab in patients with BL treated in our institute. Patients and Methods: All patients diagnosed of BL between January-2015 through December-2017 were treated in a risk stratified manner with either modified MCP-842 or modified LMB protocol. Patients with poor response to MCP 842 were shifted to LMB-salvage regimen. In addition, rituximab was given for selected patients of LMB group B or C. Result: Forty-two(49.4%) of 85 analyzed patients with BL received rituximab [Median dose:1500(Range:375-1875) mg/m2]. The incidence of febrile neutropenia(p=0.02), pneumonia(p=0.005), Intensive care unit admissions(p=0.002) and toxic deaths(p=0.04) were higher amongst BL patients who received rituximab. Pneumonia was fatal in 11 of 16(69%) patients who received rituximab. The mortality was 100% for patients who developed recurrent pneumonia after completion of treatment. On multivariate analysis, rituximab continued to be significantly associated with toxic deaths, HR:11.45(95%CI: 1.87-70.07; p=0.008). The addition of rituximab to intensive chemotherapy resulted in an inferior 1-year event free survival (49.4±8.1% vs 79.3±6.5%;p=0.025) and 1-year overall survival (63.1±8.5% vs 91.8± 4.5%;p=0.007). Also, the addition of rituximab did not improve 1-year relapse free survival (78.3±7.3% vs 83.9±6.0%;p=0.817). Conclusion: The potential immunomodulatory effect of rituximab and increased susceptibility to infections in patients from LMICs being treated under resource-constrained situations has to be carefully considered while choosing this drug in the treatment BL.