Outcome for recurrent non-Hodgkin lymphoma (NHL) in children and adolescents depends on histologic subtype. A Children's Cancer Group study (CCG-5912) was able to achieve complete remission (CR) in 40% of NHL patients. A Pediatric Oncology Group study showed a 70% response rate and 40% CR rate. Radiation therapy may have a role in treating patients who have not had a complete response to chemotherapy. All patients with primary refractory or relapsed NHL should be considered for clinical trials.
For recurrent or refractory B-lineage NHL, survival is generally 10% to 20%.[3,4,5,6,7] Chemoresistance is a major problem making remission difficult to achieve. There is no standard treatment option for patients with recurrent or progressive disease. The use of single-agent rituximab, as well as rituximab combined with standard cytotoxic chemotherapy, has shown activity in the treatment of B-cell lymphoma patients.[Level of evidence: 3iiiDii] A Children's Oncology Group (COG) study using rituximab, ifosfamide, carboplatin, and etoposide (R-ICE) to treat relapsed/refractory B-cell NHL (diffuse large B-cell lymphoma [DLBCL] and Burkitt lymphoma) showed a CR/partial remission rate of 60%.[Level of evidence: 3iiA] If remission can be achieved, high-dose therapy and stem cell transplantation may be pursued. The benefit of autologous versus allogeneic stem cell transplantation (SCT) is unclear.[5,10,11,12]; [Level of evidence: 2A] An analysis of the Center for International Blood and Marrow Transplant Research (CIBMTR) demonstrated no difference using either autologous or allogeneic donor stem cell sources, with 2 year event-free survival (EFS) to be 30% for DLBCL and 50% for Burkitt lymphoma. This analysis also showed patients not in remission at time of transplant do significantly worse.
New drugs are continually being researched and developed for Non-Hodgkin’s lymphoma. These must be shown to be safe and effective before doctors can prescribe them to patients. Through clinical trials, researchers test the effects of new drugs on a group of volunteers with non-Hodgkin’s lymphoma. Following a strict protocol and using carefully controlled conditions, researchers evaluate the investigational drugs under development and measure the ability of the new drug to treat non-Hodgkin’s lymphoma,...
For recurrent or refractory lymphoblastic lymphoma, survival in the literature ranges from 10% to 40%.[5,14]; [15,16][Level of evidence: 3iiiA] As with Burkitt lymphoma, chemoresistant disease is common. There is no standard treatment option for patients with recurrent or progressive disease. A COG phase II study of nelarabine (compound 506U78) as a single agent demonstrated a response rate of 40%. The CIBMTR analysis demonstrated that EFS was significantly worse using autologous (4%) versus allogeneic (40%) donor stem cell source, with all failures resulting from progressive disease.
For recurrent or refractory anaplastic large cell lymphoma (ALCL), 40% to 60% of patients can achieve long-term survival.[5,18,19] There is no standard approach for recurrent/refractory ALCL; standard chemotherapy, autologous SCT, and allogeneic SCT have all been employed in this setting.[5,12,18,19,20]; [Level of evidence: 2A] Several studies suggest that allogeneic SCT may result in better outcome for refractory/relapsed ALCL.[12,20] Vinblastine is active as a single agent in recurrent/refractory ALCL, inducing CR in 25 (83%) of 30 evaluable patients in one study. Nine of 25 patients treated with vinblastine alone remained in CR with median follow-up of 7 years since the end of treatment.[Level of evidence: 3iiiA]