How is advanced-stage pediatric lymphoblastic lymphoma treated?

Updated: Jun 14, 2018
  • Author: J Martin Johnston, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
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Answer

For advanced-stage lymphoblastic lymphoma, as for ALL, relatively long intervals of treatment have been most successful. The maintenance phase typically lasts 18-30 months. Protocols shorter than this have also been investigated. For example, Children's Cancer Group protocol 5941 examined an aggressive, 11-month, multiagent regimen. In this trial, the 5-year event-free survival rate was 78% ± 4.5%, and the overall survival rate was 85% ± 3.9%. These results suggest that the experimental approach is safe and is just as effective as more prolonged regimens. [64]

When results from several series were combined, patients appeared to have an excellent prognosis. Long-term survival was approximately 80%.

Despite these findings, a consensus about optimal therapy for lymphoblastic lymphoma is lacking. Treatment options include the LSA2 L2 protocol and BFM protocol 86 for non-Hodgkin lymphoma (with the reinduction phase eliminated).

Localized lymphoblastic lymphoma is unusual. In the previously mentioned BFM study, 6 of 77 subjects with non–B-cell non-Hodgkin lymphoma had stage I or II disease. Patients with localized lymphoblastic lymphoma (n=60) were included in Children's Oncology Group protocol A5971; they received a Children's Cancer Group modified version of the BFM protocol (as described above) that included reinduction but had fewer doses of intrathecal chemotherapy during the maintenance phase. Interestingly, most patients (75%) had a B-precursor phenotype. At a median follow-up of 5.9 years, the 5-year event-free survival rate was 90% (95% confidence interval, 78-96%, with an overall survival rate of 96% [84-99%]). There were no relapses among the 15 T-cell lymphoblastic lymphoma subjects. [65]

Regimens simpler than these have demonstrated comparable results. For example, protocol 77-04 from the NCI included alternating cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) and high-dose methotrexate (with leucovorin as rescue therapy). Aggressive intrathecal prophylaxis with cytarabine and methotrexate was included; local radiation therapy was not offered routinely. The total duration of therapy was 15 cycles (approximately 60 wk).


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