Outcome of children with primary resistant or relapsed non-Hodgkin lymphoma and mature B-cell leukemia after intensive first-line treatment: a population-based analysis of the Austrian Cooperative Study Group.

BACKGROUND: Children and adolescents with Non-Hodgkin lymphoma (NHL) and mature B-cell leukemia (B-ALL) have an excellent prognosis with contemporary chemotherapy stratified according to the histologic subtype and clinical stage of disease. However, a small subset of patients does not respond to front-line therapy or suffers from an early relapse.

PROCEDURE: A retrospective analysis was performed to assess the incidence, treatment, and outcome of all children with relapsed or progressed NHL and B-ALL diagnosed in Austria between 1986 and 2003 (n = 22/234).

RESULTS: Nine of 140 (6.5%) patients with B-cell NHL/B-ALL (relapse, n = 6; progress, n = 3) failed initial treatment. Four of them underwent a hematopoietic stem cell transplantation (HSCT) as second-line therapy, two patients received intensive chemotherapy alone and in three patients treatment was palliative. Eight of the nine patients died of their disease. Four of 65 (6%) patients with lymphoblastic lymphoma (LBL) (relapse, n = 2; progress, n = 2) had a treatment failure. High-dose chemotherapy followed by HSCT was performed in two of the four patients; another two patients received chemotherapy alone. Three of the four patients died of resistant disease. Nine of 29 (31%) patients with anaplastic large cell lymphoma (ALCL) (relapse, n = 7; progress, n = 2) failed first-line therapy. Six underwent a HSCT (autologous, n = 3; allogeneic, n = 3) and are currently in second complete remission. Treatment of the other three patients consisted of chemotherapy alone-they all died of tumor progression.

CONCLUSIONS: Conclusively, patients with early relapsed and progressive B-cell neoplasia or LBL have a very poor prognosis with current treatment approaches, while those with ALCL have a respectable chance to achieve a sustained complete second remission with high-dose chemotherapy and HSCT.