Small noncleaved, non-Burkitt's (Burkitt-like) lymphoma: Cytogenetics predict outcome and reflect clinical presentation

Purpose: To correlate cytogenetic abnormalities with clinical presentation and outcome in Burkitt-like, small noncleaved nan-Burkitt's lymphoma (SNC-N B), Patients and Methods: Thirty-nine patients with SNC-NB lymphoma and a clona l karyotype were evaluated between January 1989 and January 1996, All were from British Columbia, Canada, underwent uniform clinical staging, and were treated on investigational protocols by a small group of clinicians. Results: Three groups of patients were identified by clonal karyotype on cy togenetic analysis: (1)those with a c-myc translocation (n = 11); (2) those with dual translocation of c-myc and bcl-2 (n = 13); and (3) those with ot her cytogenetic abnormalities (n = 15), The c-myc group was younger, presen ted with earlier stage de novo disease, and had a better clinical prognosti c factor profile, The dual-translocation and other groups were older and pr esented in advanced stage with poorer prognostic features, and a larger pro portion of the dual-translocation group patients had transformed from previ ously diagnosed follicular lymphoma, The median overall survival (OS) time for all patients wets 5 months, The median OS time for the dual-translocati on group was only 2.5 months, as compared with 7 months and 8 months for th e c-myc and other group, respectively (P <.001), There were no survivors be yond 7 months among the dual-translocation group, as opposed to 32% and 25% 2-year OS Kites in the c-myc and other group. Conclusion: SNC-NB lymphoma is a clinically and cytogenetically heterogenou s disease. Dual translocation of c-myc and bcl-2 is characterized by a rapi d clinical course and extremely poor outcome. This latter entity may repres ent the most clinically aggressive lymphoma thus far characterized and warr ants intensive investigational treatment where feasible, J Clin Oncol 17:15 58-1567, (C) 1999 by American Society of Clinical Oncology.