Large cell non-Hodgkin lymphoma of childhood : Analysis of 78 consecutive patients enrolled in 2 consecutive protocols at the Memorial Sloan-Kettering Cancer Center
✍ Scribed by Jaume Mora; Daniel A. Filippa; Howard T. Thaler; Tatyana Polyak; Milicent L. Cranor; Norma Wollner
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 130 KB
- Volume
- 88
- Category
- Article
- ISSN
- 0008-543X
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✦ Synopsis
The authors report a study of pediatric patients with advanced diffuse large cell lymphoma (DLCL) who were treated with 2 consecutive regimens, LSA2-L2 and LSA4, over a 25-year-period at the Memorial Sloan-Kettering Cancer Center. They also describe a comparative analysis of two subgroups retrospectively identified as having CD30 positive (ϩ) anaplastic large cell lymphoma (ALCL) and CD30 negative (Ϫ) DLCL. To the authors' knowledge, this study represents the longest follow-up on the largest series of uniformly treated pediatric DLCL patients reported to date.
METHODS.
A total of 78 consecutive patients were treated for Stage III/IV DLCL.
Immunophenotypic data were obtained retrospectively for 52 patients using 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 stage 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 months (range, 24 -312 months). The recurrence rate was significantly higher in the CD30ϩ ALCL subgroup (33%) than in the CD30Ϫ 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 CD30ϩ (36.5%), 18 had disease of T-cell phenotype, and 1 had disease of B-cell lineage.
CONCLUSIONS.
The CD30Ϫ 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 CD30ϩ ALCL group; although these tumors were of T-cell lineage and had a significantly higher rate of late recurrences (median follow-up of 24 months) they all were salvageable. Based on the findings of the current study, the authors propose that T-cell CD30ϩ 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 lymphomas; and no central nervous system prophylaxis beyond the induction period unless other recognized risk factors are present.