T-cell rich B-cell lymphoma: clinical distinctiveness and response to treatment in 45 patients

T-cell rich B-cell lymphoma (TCR-BCL) is a recently described pathologic diagnosis without a place among traditional lymphoma classification systems. In the past, TCR-BCL has been included among other diagnoses, in particular lymphocyte predominant Hodgkin's disease (LPHD). The study of TCR-BCL cohorts may elucidate clinical distinctiveness, response to therapy, and the effect of treatment regimen on outcome. Between 1992 and 1997, a hematopathologist at Memorial Sloan-Kettering Cancer Center (MSKCC) diagnosed 45 patients with TCR-BCL according to published criteria. Clinical data was collected through retrospective chart review and communication with other patient providers. Our patients presented most commonly as males in their fourth decade with advanced stage disease. Three-year overall survival (OS) and failure-free survival (FFS) were 73 and 37%, respectively. Conventional combination chemotherapy regimens were utilized for an aggressive non-Hodgkin's lymphoma (NHL) diagnosis in 26 and for a Hodgkin's disease (HD) diagnosis in 10. Disease-free survival (DFS) was significantly better for NHL (36%) vs. HD (10%) directed chemotherapy at 3 years (p = 0.003). Overall survival at 3 years was not statistically different (62 vs. 79%) due to successful salvage therapy in both groups. It is important to distinguish TCR-BCL from LPHD and classical HD. Advanced stage, extranodal disease, involvement of the mediastinum, mesentery and/or spleen are clinical clues to a TCR-BCL diagnosis. Chemotherapy directed to a NHL diagnosis rather than HD results in a significant improvement in disease-free survival. Initial Hodgkin's disease-directed (HD-directed) chemotherapy should be avoided, although salvage transplantation may result in prolonged survival.