Introduction

One of the great success stories of medical oncology has been the treatment of acute lymphoblastic leukemia (ALL) in children. Through a strong cooperative group effort, the recruitment of a high proportion of afflicted patients, and the conduct of multiple randomized trials, there have been successive improvements in treating childhood ALL, so that now 60-75% of the children with ALL can achieve long-term disease-free survival.1 Unfortunately, adults with ALL do not fare as well. Despite complete remissions (CRs) in 60-90% , long-term disease-free survival still remains at 20-40%.2,3 In addition to deaths (5-10%) during remission induction, approximately 10-25% of patients have resistant disease. Even for patients who achieve a CR, 60-70% will relapse, often within 2 years of achieving the remission (a time when most patients are still receiving maintenance therapy). Management of both primary refractory and relapsed patients poses a great challenge to clinicians; it occurs commonly, and long-term survival in these patients is typically poor. Newer treatment strategies and novel agents are urgently needed to improve the prognosis for these patients. This chapter will review some of the prognostic features that influence the likelihood of failing to achieve a CR, chemotherapy strategies often employed in the salvage setting, stem cell transplantation, and special cases, including patients with Philadelphia (Ph)-chromosome disease, patients with central nervous system (CNS) relapse, and the use of newer agents.

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