LARGE-CELL NON-HODGKIN-LYMPHOMA OF CHILDHOOD CLINICAL CHARACTERISTICSAND OUTCOME

Less is known about the clinical features and treatment outcome in ped iatric large cell non-Hodgkin lymphoma (NHL) than the lymphoblastic an d small noncleaved cell subtypes of NHL. To characterize presenting fe atures and assess possible risk factors associated with this diagnosis , we analyzed data for 91 patients treated on a succession of multiage nt regimens from 1975 to 1990. Five-year event-free survival (EFS) (+/ -SE) was related to disease extent (St Jude system): stage I (n = 24), 95%+/-5%; stage II (n = 20), 84%+/-9%; stage III (n = 38), 50%+/-10%; and stage IV (n = 9), 22%+/-11%. Advanced stage disease, age less tha n or equal to 5 years and serum LDH > 500 U/l were associated with poo rer EFS in the univariate model (p < 0.001, 0.005, and 0.002, respecti vely). In the multivariate model, advanced stage and age retained prog nostic significance (p = 0.001 and 0.02, respectively), but LDH did no t. Among limited stage cases, age less than or equal to 5 years was th e only adverse risk feature (p = 0.016); treatment era (pre- vs. post- 1979) was the only significant feature in patients with advanced disea se (p = 0.004). Intrathoracic primaries were associated with a better outcome than other sites among the 38 stage III patients (p = 0.005). Only one of eight patients with bone marrow disease remains failure-fr ee. The excellent results for limited stage pediatric large cell NHL p ermit consideration of treatment modifications to decrease toxicity; f or cases with advanced disease, especially those with bone marrow invo lvement, novel therapeutic approaches are clearly needed.