Pediatric mature B-cell non Hodgkin lymphoma treatment with LMB-96 protocol. The Children Cancer Hospital Egypt experience

Abstract

Original ArticleAbstract Purpose: Burkitt lymphoma (BL) is a highly aggressive mature B-cell non-Hodgkin lymphoma (NHL) and is the fastest growing human tumor. The outcome of childhood NHL has improved steadily over the past decades through the use of intensive sequential multi-agent chemotherapy regimens. Methods: A retrospective study having all patients 18 years old or younger diagnosed with mature B cell NHL and treated at Children Cancer Hospital Egypt (CCHE). All children were treated according to the modified (LMB 96) protocol during the period between July 2007 and December 2012. Patients were followed up till June 2013. Results: Three hundred and seventy-seven patients were diagnosed with mature B cell NHL and received the LMB96 treatment protocol. The majorities were males (76.4%) with a median age of 5.3 years, and ranged from 0.1-18.0 years. The median follow-up period was 28.2 months (range 0.9-72 months). Burkitt lymphoma was the most predominant pathologic subtype (79.6%, n = 300), and abdominal mass as a primary site was the most common presentation (71.3%). Twenty seven patients (7.2%) were treated as group A, 268 (71.0%) as group B, and 82 (21.8%) patients as high risk group C. Seventy-one (18.8%) patients suffered adverse events. Major adverse events were early deaths in 17 patients (4.5%), death during induction chemotherapy seen in 18 patients (4.7%), and during maintenance therapy in 7 patients (1.8%), tumor progression in 19 patients (5.0%), and relapse in 10 patients (3.7%). Sixty-three patients (16.7%) died during the study period. The main causes of death were tumor lysis syndrome (TLS) in 25.3%, and severe sepsis during chemotherapy in 41.3% of the patients. The 3 years OS and EFS were 83.3% and 80.4% respectively for the whole groups of patients. OS and EFS were 100% for group A, and 87.5%±3.9% and 85.9±4.3% for group B. For group C BM + /CNS -patients, OS was 55.62%±15.8%, and EFS of 53.8%±15.6%. For BM + /CNS + patients, OS and EFS were 63.2%±21.76% and 57.9%±22.1% respectively. BM -/CNS + patients had OS 72.4%±18.8% and EFS 67.6%±19.7% at 36 months. Conclusion: TLS and chemotherapy related toxicity remains a major challenge affecting the outcome of pediatric mature B cell NHL. We identified bone marrow involvement as a risk factor affecting treatment outcome. Aggressive supportive care measures are mandatory to avoid unacceptable high toxicity related mortality.

Temporal trends in childhood cancer survival in Egypt, 2007 to 2017: A large retrospective study of 14 808 children with cancer from the Children's Cancer Hospital Egypt

“…In MDS and JMML, limited capacity to perform BMT to all patients was the main reason for inferior survival 57 . Inferior survival of NHL was due to late referrals, high tumor burden, 49 higher percent of TRMs than in HICs and poor survival of Burkitt lymphoma 58 . For CNS tumors, lower survival is likely due to delayed diagnosis, 49 lack of second‐line treatment (resurgery/BMT) for recurrent patients, unlike HICs who give multimodality treatment strategies.…”

“…57 Inferior survival of NHL was due to late referrals, high tumor burden, 49 higher percent of TRMs than in HICs and poor survival of Burkitt lymphoma. 58 For CNS tumors, lower survival is likely due to delayed diagnosis, 49 We believe that focusing our efforts on the childhood cancers with significantly inferior survival at CCHE, especially those with large volume of patients and large gap (>10%) in survival, along with other priority areas would most likely improve 5-year survival >80%, similar to HICs. 13,16,34,41 Accordingly, we suggest key recommendations in current practice, resource planning and public health issues to overcome current barriers to optimal care and find innovative and strategic solutions to improve survival of children with cancer in Egypt.…”

“…57 Inferior survival of NHL was due to late referrals, high tumor burden, 49 higher percent of TRMs than in HICs and poor survival of Burkitt lymphoma. 58 For CNS tumors, lower survival is likely due to delayed diagnosis, 49 lack of second-line treatment (resurgery/BMT) for recurrent patients, unlike HICs who give multimodality treatment strategies. In children with solid tumors, poor survival is due to delayed referrals, 49 advanced stage at diagnosis, delays in treatment and limited salvage therapy options.…”

Neurologic Complications in Patients With Lymphoreticular Malignancy: A Descriptive Cohort Study

“…Patients with Hodgkin lymphoma (HL) were treated according to InPOG-HL-protocol consisting of nonintensive chemotherapy 12 . LMB-96 protocol was used to treat patients with Burkitt lymphoma and diffuse large B-cell lymphoma, and International protocol for the treatment of childhood Anaplastic large cell lymphoma (ALCL 99) was used to treat patients with anaplastic large cell lymphoma; both consisting of intensive chemotherapy 13 …”

“…12 LMB-96 protocol was used to treat patients with Burkitt lymphoma and diffuse large B-cell lymphoma, and International protocol for the treatment of childhood Anaplastic large cell lymphoma (ALCL 99) was used to treat patients with anaplastic large cell lymphoma; both consisting of intensive chemotherapy. 13 Platelet transfusion was given to children with bleeding manifestations at any platelet count and for platelet count <20,000/mm 3 even if asymptomatic. The children in our study received only cotrimoxazole prophylaxis; no other antibiotic or antifungal prophylaxis was used.…”