Peripheral T-cell lymphoma in children and adolescents: a single-institution experience.

BACKGROUND

Peripheral T-cell lymphoma (PTCL) is rare in pediatric patients and is associated with worse outcome compared with other pediatric non-Hodgkin lymphomas. We report our institutional experience over a 10-year period.

METHODS

Patients were identified through the institutional oncology database. Data were abstracted through a detailed retrospective review of patient charts. Those with isolated cutaneous T-cell lymphoma were excluded from this analysis.

RESULTS

Thirteen patients were diagnosed with PTCL during the 10-year period. All presented with advanced disease. According to the WHO PTCL classification, 7 patients had PTCL not otherwise specified, 3 had hepatosplenic T-cell lymphoma and 3 had T-cell posttransplant lymphoproliferative disease. Six of the patients had previously received or were receiving immunosuppressive therapy at the time of their diagnosis. Patients were treated with various chemotherapeutic regimens, including B-cell non-Hodgkin lymphomas and T-cell acute lymphoblastic leukemia-like therapy. Patients who had refractory or recurrent disease were changed to alternative therapy. Eight patients underwent stem cell transplantation, 7 allogeneic and 1 autologous. Of the 13 patients, 7 died. Three patients died from disease progression, 3 died from treatment-related mortality, and 1 patient died from cardiac rejection. Six patients (46%) are alive and disease free at a median of 21 months (2 to 79 mo).

CONCLUSIONS

Children with PTCL have an unfavorable outcome. A standard approach to management of pediatric PTCL needs to be established to improve outcome. Because of the rarity of this condition, this will require collaborative studies.