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.
Lymphoma refers to a malignancy of the lymphatic system. The lymphatic system is a network of nodes (knots of tissue) connected by vessels. Together, the lymph nodes drain fluid and waste products from the body. The lymph nodes act as tiny filters, removing foreign organisms and cells.
Lymphocytes, are a type of white blood cell that helps fight infections caused by bacteria, viruses, or fungi. The lymph node function is to prevent infections from entering the bloodstream. When the lymphatic...
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]