Hematopoietic stem cell transplantation for pediatric patients with non-anaplastic peripheral T-cell lymphoma. An EBMT pediatric diseases working party study

Abstract

Peripheral T-cell lymphomas (PTCL) other than anaplastic large-cell lymphoma are rare in children, and the role of hematopoietic stem cell transplantation (HSCT) has not been clarified yet. In a retrospective analysis of registry-data of the European Society for Blood and Marrow Transplantation we analyzed 55 patients aged < 18 years who received allogeneic (N = 46) or autologous (N = 9) HSCT for PTCL. Median age at HSCT was 13.9 years; 33 patients (60%) were in first remission, and 6 (19%) in progression at HSCT. Conditioning was myeloablative in 87% of the allogeneic HSCTs and in 27 (58.7%) based on total body irradiation. After allogeneic HSCT the 5-year overall- and progression-free survival was 58.9% (95% CI 42.7-71.9) and 52.6% (95% CI 36.8-66.1), respectively. 5-year relapse incidence was 27.6% (95% CI 15.1-41.6), the non-relapse mortality rate was 19.8% (95% CI 9.7-32.6). Five of the six patients with progression at HSCT died. Seven of nine patients after autologous HSCT were alive and disease-free at last follow-up. Our data suggest a role of allogeneic HSCT in consolidation-treatment of patients with high-risk disease, who reach at least partial remission after primary- or relapse-therapy, whereas patients with therapy-refractory or progressive disease prior to transplantation do not profit from HSCT.

Fig. 1

Study population chart. Numbers of patients screened from the EBMT-database for inclusion in the present study.

Fig. 2. Study population chart Numbers of patients screened from the EBMT-database for inclusion in the present study.

Outcome estimates after allogeneic HSCT: a Kaplan-Meier estimates of overall survival (OS). The 2-year OS was 66.8% (95% CI 51.1–78.5), the 5-year OS was 58.9% (95% CI 42.7–71.9), respectively. b Probability of progression-free survival (PFS): The 2-year PFS was 55.3% (95% CI 39.6-68.5), the 5-year PFS was 52.6% (95% CI 36.8-66.1), respectively. c Cumulative relapse incidence (RI): 2-year cumulative RI was 24.9% (95% CI 13.2–38.4), 5-year cumulative RI was 27.6% (95% CI 15.1–41.6), respectively. d Estimate of non-relapse mortality (NRM): the 2-year and 5-year NRM was 19.8% (95% CI 9.7–32.6).