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

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Abstract
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 intensived 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.

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