Managing CNS disease in adults with acute lymphoblastic leukemia

The central nervous system (CNS) is an important site of involvement by acute lymphoblastic leukemia (ALL) in adults. The prevalence is sufficiently high that prophylactic treatment is routinely given to this sanctuary site in order to eradicate occult disease that might otherwise lead to a relapse. A lumbar puncture should be routinely performed in all newly diagnosed patients with ALL. The risks of CNS leukemia vary by phenotype and genotype. Preventive treatment of the CNS during post-remission therapy has become an integral part of all current ALL treatment protocols. Most treatment regimens combine multiple doses of intrathecal chemotherapy with high-dose systemic methotrexate and/or cytarabine. Cranial irradiation is less commonly used for prophylaxis but is still the most effective treatment for overt CNS leukemia. Recurrences within the CNS usually coincide with or predict soon afterwards for systemic relapse in the marrow and blood.