Background: Patients with T-cell acute lymphoblastic leukemia (T-ALL) frequently present with unfavorable features at diagnosis. Therefore, they are considered to have a higher risk to relapse. We sought to correlate initial central nervous system (CNS) disease at diagnosis with shortened survival in childhood T-ALL.
Procedure: A retrospective analysis of 48 children with T-ALL was performed. The group consisted of 32 boys and 16 girls whose median age was 8 years. Their CNS status was classified as CNS-1 (no blast cells in cerebrospinal fluid (CSF); n = 44), CNS-2 (<5 WBC/microl of CSF with blast cells; n = 0), or CNS-3 (> or =5 WBC/microl of CSF with blast cells or signs of CNS involvement; n = 4). For univariate prognostic analyses, we used the log-rank test to determine the influence of patient characteristics (age, sex, lymphomatous presentations, initial leukocyte count, CNS disease, and newer therapeutic strategies) on each point.
Results: Complete remission was induced in 87.5% of patients. Median survival was 37 months, and 5-year overall survival and disease-free survival rates were 49.5% +/- 8.1% and 47.1% +/- 8.2%, respectively. Patients without initial CNS involvement seemed to have a trend toward longer overall survival (P = 0.036). Disease-free survival was not influenced by age, leukocyte count, or other factors analyzed.
Conclusions: Patients who present with initial CNS involvement have a prognosis worse than that of patients without CNS disease. The introduction of early and effective CNS-directed therapy might no longer portend a poor prognosis for CNS leukemia.