Long-term efficacy of CHOP-based chemotherapy alone or combined with radiotherapy for adolescent/childhood primary systemic anaplastic large-cell lymphoma

Objective: To investigate the long-term efficacy of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone)-based chemotherapy alone or combined with regional radiotherapy for adolescent/childhood primary systemic anaplastic large-cell lymphoma (ALCL). Methods: A retrospective analysis was performed on the medical records of 28 patients not older than 21 years with a confirmed diagnosis of primary systemic ALCL, who were admitted to our hospital from January 1998 to December 2010. Of 12 stage I/II patients, 2 received chemotherapy alone, and 10 received chemotherapy and radiotherapy; of 16 stage III/IV patients, 14 received chemotherapy alone, and 2 received chemotherapy and radiotherapy. CHOP regimen was adopted in 15 patients, and CHOP regimen combined with other high-intensity regimens was adopted in 13 patients. There were 3-17 chemotherapy cycles (median: 6 cycles). Radiotherapy was performed mainly by involved field radiation, with a dose of 39.6-50.0 Gy (median: 45 Gy). Results: After the first course of treatment, 25 (89%) of all patients achieved a complete remission (CR), and 3 patients experienced disease progression. With a median follow-up of 45.3 months, the 5-year event-free survival (EFS) and overall survival (OS) for all patients were 80% and 93%, respectively. The 5-year OS was 100% for patients achieving a CR after treatment, versus 0% for those not achieving a CR (P = 0.000). The 5-year EFS was 38% for patients with involvement of #>=# 2 extranodal organs, versus 85% for those with involvement of < 2 extranodal organs (P = 0.010). Conclusions: CHOP chemotherapy combined with regional radiotherapy (for patients with localized disease) is an effective treatment for adolescent/childhood primary systemic ALCL, but long-term follow-up is needed to investigate treatment-related toxicities. (authors)