Background: Even though rituximab has emerged as standard of care for the management of high-risk pediatric Burkitt lymphoma (BL), its safety in children from the lowmiddle-income countries (LMICs) remains to be proven. We herein report our experience of using rituximab in children with BL. Methods: All patients diagnosed with BL between January 2015 and December 2017 were treated in a risk-stratified manner with either the modified MCP-842 or modified LMB protocol. Patients with poor response to MCP-842 were switched to the LMBsalvage regimen. In addition, rituximab was given to selected high-risk patients. Result: Forty-two (49.4%) of 85 patients with BL received rituximab. The incidence of febrile neutropenia (90.5% vs 67.4%; P = 0.02), pneumonia (38.1% vs 11.6%; P = 0.005), intensive care unit admissions (54.5% vs 17.6%; P = 0.002), and toxic deaths (26.2% vs 9.3%; P = 0.04) was higher among BL patients who received rituximab. Pneumonia was fatal in 11 of 16 (69%) patients who received rituximab. On multivariate analysis, rituximab continued to be significantly associated with toxic deaths (OR: 11.45 [95% CI: 1.87-70.07; P = 0.008]). The addition of rituximab to intensive chemotherapy resulted in an inferior one-year event-free survival (49.4% ± 8.1% vs 79.3% ± 6.5%; P = 0.025) and one-year overall survival (63.1% ± 8.5% vs 91.8% ± 4.5%; P = 0.007) with no improvement in one-year relapse-free survival (78.3% ± 7.3% vs 83.9% ± 6.0%; P = 0.817). Conclusion: Rituximab was associated with increased toxicities and toxic deaths in our patients. The potential immunomodulatory effect of rituximab and increased susceptibility to infections in patients from LMICs have to be carefully considered while choosing this drug in the treatment of BL in resource-constrained settings.
Background
Not all the significant progress made in the management of children with hepatoblastoma (HB) has translated into improved outcomes in limited‐resource settings. There are limited data on outcomes in children with HB from India.
Methods
All patients diagnosed with HB between July 2013 and December 2020 were risk‐stratified and treated as per International Liver Tumor Strategy Group (SIOPEL). Patients with standard‐risk HB received cisplatin monotherapy and those with high‐risk HB received alternating cycles of cisplatin and the combination of carboplatin plus doxorubicin. Data regarding demographic details, chemotherapy, surgery, liver transplantation, outcomes, prognostic factors, and toxicity were collected.
Results
Of 157 patients with HB, 117 (74%) were high risk, 31 (20%) were standard risk, and nine (6%) unknown. Patients with standard‐risk disease had excellent outcomes, with 3‐year event‐free survival (EFS) and overall survival (OS) of 96% and 100%, respectively. Among high‐risk HB, six underwent orthotopic liver transplantation of which four were alive at last follow‐up. The 3‐year EFS and OS of patients with high‐risk disease was 56% and 66%, respectively. Outcomes of patients with PRETEXT IV (3‐year EFS: 42%, 3‐year OS: 50%) and metastatic disease (3‐year EFS: 30%, 3‐year OS: 50%) were dismal. Patients with serum alpha‐fetoprotein (AFP) reduction greater than 90% following two courses of chemotherapy had favorable outcomes; 3‐year EFS: 80% versus 58% (p = .013) and 3‐year OS: 95% vs. 68% (p < .01). Only two (6%) of 31 patients with relapse/refractory HB were alive at a median follow‐up of 36 months, and both had received salvage chemotherapy and surgery.
Conclusions
While children with standard‐risk HB had excellent outcomes, those with high‐risk disease continue to do poorly. Serial monitoring of serum AFP values is a cost‐effective and reliable predictor of outcomes. Orthotopic liver transplantation remains a viable option for inoperable disease in resource‐limited settings as well.
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