Revised: May 31, 2014
Accepted: June 18, 2014
Published online: August 6, 2014
Recurrent acute lymphoblastic leukaemia (ALL) is a common disease for pediatric oncologists and accounts for more deaths from cancer in children than any other malignancy. Although most patients achieve a second remission, about 50% of relapsed ALL patients do not respond to salvage therapy or suffer a second relapse and most children with relapse die. Treatment must be tailored after relapse of ALL, since outcome will be influenced by well-established prognostic features, including the timing and site of disease recurrence, the disease immunophenotype, and early response to retrieval therapy in terms of minimal residual disease (MRD). After reinduction chemotherapy, high risk (HR) patients are clear candidates for allogeneic stem cell transplantation (SCT) while standard risk patients do better with conventional chemotherapy and local therapy. Early MRD response assessment is currently applied to identify those patients within the more heterogeneous intermediate risk group who should undergo SCT as consolidation therapy. Recent evidence suggests distinct biological mechanisms for early vs late relapse and the recognition of the involvement of certain treatment resistance related genes as well cell cycle regulation and B-cell development genes at relapse, provides the opportunity to search for novel target therapies.
Core tip: Selected recent publications regarding the current management of childhood relapsed acute lymophoblastic leukemia have been reviewed. Controversies, current lines of investigation and possible future directions are discussed.