Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Recurrent Childhood NHL Treatment
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 and Burkitt lymphoma) showed a CR/partial remission (PR) rate of 60%.[Level of evidence: 3iiA] If remission can be achieved, high-dose therapy and stem cell transplantation (SCT) may be pursued. The benefit of autologous versus allogeneic SCT is unclear.[5,10,11,12]; [Level of evidence: 2A]; [Level of evidence: 3iiiDii] (Refer to the PDQ summary on Childhood Hematopoietic Cell Transplantation for more information about transplantation). An analysis of the Center for International Blood and Marrow Transplant Research (CIBMTR) data demonstrated no difference using either autologous or allogeneic donor stem cell sources, with 2-year event-free survival (EFS) to be 30% for diffuse large B-cell lymphoma and 50% for Burkitt lymphoma. This analysis also showed patients not in remission at time of transplant do significantly worse.[12,13] For patients who have a second relapse after initial autologous SCT, an allogeneic SCT was found to be a promising treatment in a study of adults with diffuse large B-cell lymphoma.
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For recurrent or refractory lymphoblastic lymphoma, survival in the literature ranges from 10% to 40%.[5,16]; [17,18][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, 40% to 60% of patients can achieve long-term survival.[5,20,21] There is no standard approach for recurrent/refractory anaplastic large cell lymphoma; standard chemotherapy, followed by autologous SCT or allogeneic SCT, if remission can be achieved, have all been employed in this setting.[5,12,20,21]; [Level of evidence: 2A] In a retrospective study of relapsed or refractory anaplastic large cell lymphoma in patients who received Berlin-Frankfurt-Muenster–type first-line therapy, reinduction chemotherapy followed by autologous stem cell transplant resulted in 59% 5-year EFS and 77% overall survival.[Level of evidence: 2A] However, outcome of patients with bone marrow or central nervous system involvement, relapse during first-line therapy, or CD3-positive anaplastic large cell lymphoma was poor. These patients may benefit from allogeneic transplantation. Several additional studies suggest that allogeneic SCT may result in better outcome for refractory/relapsed anaplastic large cell lymphoma.[12,22] Vinblastine is active as a single agent in recurrent/refractory anaplastic large cell lymphoma, 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]