The importance of an isolated central nervous system relapse in children with acute lymphoblastic leukemia.

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Abstract
The influence of an initial isolated meningeal relapse on treatment outcome in 839 children with acute lymphoblastic leukemia (ALL) who were admitted to St Jude Children''s Research Hospital (Memphis) from mid-1967-mid-1979 was assessed. The patients were entered in a series of 5 clinical trials (total therapy studies V-IX), each designed to test 1 or more modifications of treatment for ALL. Two groups were compared: 699 children who received CNS prophylaxis (2,400-rad craniospinal irradiation or 2400-rad cranial irradiation plus intrathecal methotrexate) vs. 56 who did not. With a time-dependent covariate model and a matching technique, a 2- to 3.5-fold increase was apparently indicated in the risk of hemotologic relapse or death among patients who experienced an isolated CNS relapse compared with similar patients (matched for leukocyte count and length of complete remission) who remained free of CNS involvement. Of the 107 children with an initial isolated CNS relapse, 89 (83%) have died or have had a subsequent relapse. There was no detectable difference in the rate of hematologic relapse or death after a CNS relapse between patients who had received preventive therapy and those who had not. CNS prophylaxis apparently important both for the prevention of initial CNS leukemia and for reducing the risk of hematologic relapse or death subsequent to a CNS relapse.