Acute lymphoblastic leukemia. A comprehensive review with emphasis on biology and therapy.

BACKGROUND

Acute lymphoblastic leukemia (ALL) is the most common malignancy in children. It is now curable in 60-70% of children. Most of the current understanding of the biology and treatment of ALL originates from studies of children. In adults, although much progress has been achieved, ALL is curable in only 20-35% of patients.

METHODS

A review of the biology and treatment of ALL from the English literature was performed.

RESULTS

Immunophenotypic and cytogenetic analyses of ALL have contributed to a more rational classification of ALL. These analyses have identified subgroups with poor prognosis or with different therapeutic requirements. Overall, 60-70% of adults with ALL have poor prognostic features, including older age, a high leukocyte count, non-T-cell immunophenotype, Ph-positive genotype, and longer time to achieve a complete remission. These patients have a cure rate of 20-25%, whereas those without these risk factors, have a 60-70% probability of survival. The use of more intensive induction regimens with growth factor support may improve survival rates. Also, intensive consolidation-intensification may improve survival rates. Most patients benefit from maintenance therapy, but the dose schedule must be optimized. Central nervous system (CNS) prophylaxis is beneficial, particularly for patients with a high risk for CNS relapse and when introduced early during induction of remission. Patients with high risk characteristics may benefit from allogeneic bone marrow transplantation (BMT) during first remission, and all other patients may benefit from it during first or subsequent relapse. Autologous BMT may be a valuable option for poor compliant patients.

CONCLUSIONS

Although the prognosis of patients with ALL has improved markedly during the past decades, newer strategies, including more dose-intensive therapy, the search for new drugs, and more target-specific therapy, are needed to improve the current cure rates.