Fm. Uckun et al., IMPROVED CLINICAL OUTCOME FOR CHILDREN WITH T-LINEAGE ACUTE LYMPHOBLASTIC-LEUKEMIA AFTER CONTEMPORARY CHEMOTHERAPY - A CHILDRENS CANCER GROUP-STUDY, Leukemia & lymphoma, 24(1-2), 1996, pp. 57-70
The prognostic importance of T-lineage acute lymphoblastic leukemia (A
LL) in contemporary programs of intensive chemotherapy has been contro
versial. We therefore assessed the impact of this biological feature i
n risk-adjusted frontline chemotherapy studies of the Children's Cance
r Group (CCG), conducted from 1983 to 1994. A substantially greater pr
oportion of T-lineage patients (N = 730) presented with poor-risk feat
ures as compared to B-lineage patients (N = 3668) treated in the same
studies (71.1% vs. 39.7%, P < 0.0001). Consequently, in the CCG-100 se
ries of clinical trials (1983-1989), which tested regimens that were l
argely of moderate intensity, T-lineage ALL patients had an excess of
adverse early events compared to patients in the B-lineage group: 3-ye
ar event-free survival (EFS) estimate, 65.8% vs 78.2% (P < 0.0001). Wi
th the introduction of more intensive chemotherapy in studies from 198
9 to 1994 (CCG-1800 series), we observed a progressive and significant
improvement in the clinical outcome of patients with T-lineage immuno
phenotype. Three- and 5-year EFS probabilities increased from 65.8% to
78.1% and from 61.0% to 75.2%, respectively, becoming comparable to o
r slightly better than results for B-lineage ALL patients. When adjust
ed for the competing effects of leukocyte count, age, organomegaly and
other poor-risk features, T-lineage immunophenotype showed no importa
nt impact on the overall EFS pattern. These findings demonstrate the l
oss of adverse prognostic influence by T-lineage ALL in a large progra
m of intensive chemotherapy developed over the past decade.