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-NB), Patients and Methods: Thirty-nine patients with SNC-NB lymphoma and a clonal 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 cytogenetic 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 other cytogenetic abnormalities (n = 15), The c-myc group was younger, presented with earlier stage de novo disease, and had a better clinical prognostic factor profile, The dual-translocation and other groups were older and presented in advanced stage with poorer prognostic features, and a larger proportion of the dual-translocation group patients had transformed from previously diagnosed follicular lymphoma, The median overall survival (OS) time for all patients wets 5 months, The median OS time for the dual-translocation group was only 2.5 months, as compared with 7 months and 8 months for the c-myc and other group, respectively (P <.001), There were no survivors beyond 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 heterogenous disease. Dual translocation of c-myc and bcl-2 is characterized by a rapid clinical course and extremely poor outcome. This latter entity may represent the most clinically aggressive lymphoma thus far characterized and warrants intensive investigational treatment where feasible, J Clin Oncol 17:1558-1567, (C) 1999 by American Society of Clinical Oncology.