Large cell non-Hodgkin lymphoma of childhood - Analysis of 78 consecutive patients enrolled in 2 consecutive protocols at the Memorial Sloan-Kettering Cancer Center

BACKGROUND. The authors report a study of pediatric patients with advanced diffuse large cell lymphoma (DLCL) who were treated with 2 consecutive regi mens, LSA2-L2 and LSA4, over a 25-year-period at the Memorial Sloan-Ketteri ng Cancer Center. They also describe a comparative analysis of two subgroup s retrospectively identified as having CD30 positive (+) anaplastic large c ell lymphoma (ALCL) and CD30 negative (-) DLCL. To the authors' knowledge, this study represents the longest follow-up on the largest series of unifor mly treated pediatric DLCL patients reported to date. METHODS. A total of 78 consecutive patients were treated for Stage III/IV D LCL. Immunophenotypic data were obtained retrospectively for 52 patients us ing a panel of monoclonal antibodies against CD30, CD15, CD45, CD45Ro, CD43 , epithelial membrane antigen, CD5, BCL-2, cyclin-D, and p53. RESULTS. A disease free survival rate of 72% in patients with advanced stag e DLCL using the LSA2-L2 and LSA4 regimens. Of the 78 treated patients, 56 are alive and without evidence of disease with a median follow-up of 120 mo nths (range, 24-312 months). The recurrence rate was significantly higher i n the CD30+ ALCL subgroup (33%) than in the CD30- DLCL group (0.04%). Of 52 patients for whom immunophenotypic data were available, 28 had disease of B-cell lineage, 24 had disease of T-cell/null phenotype, 19 were CD30+ (36. 5%), 18 had disease of T-cell phenotype, and 1 had disease of B-cell lineag e. CONCLUSIONS,The CD30- DLCL cases mostly were of B-cell lineage, had a small risk of treatment failure, and did not develop a recurrence off therapy. A distinct clinical pattern was identified for the CD30+ ALCL group; althoug h these tumors were of T-cell lineage and had a significantly higher rate o f late recurrences (median follow-up of 24 months) they all were salvageabl e. Based on the findings of the current study, the authors propose that T-c ell CD30+ ALCL be addressed in the future according to equal dose intensity regimens in induction therapy, as is done for B-cell lymphomas; prolonged periods of maintenance chemotherapy, as is done for T-cell lymphoblastic ly mphomas; and no central nervous system prophylaxis beyond the induction per iod unless other recognized risk factors are present. Cancer 2000;88:186-97 . (C) 2000 American Cancer Society.