Survival of patients with CD20-negative variants of large B-cell lymphoma: an analysis of the National Cancer Data Base

Using records from the National Cancer Data Base, we studied overall survival of CD20-negative variants of diffuse large B-cell lymphoma (DLBCL): primary effusion (PEL, N=228), plasmablastic (PBL, N=481), ALK-positive large B-cell (ALK+LBLC, N=15), and human herpesvirus-8-positive DLBCL (HHV8+DLBCL, N=77). Three-year survival was 27% for PEL, 40% for PBL, 34% for ALK+LBCL, and 63% for HHV8+DLBCL. Compared with unspecified DLBCL, and adjusting for clinical characteristics (including the HIV status), survival was significantly worse for PEL (hazard ratio [HR], 1.58; 95%confidence interval [CI], 1.31-1.90), PBL (HR 1.66; 95%CI, 1.41-1.95), and ALK+LBCL (HR, 2.70; 95%CI, 1.27-5.75), but not for HHV8+DLBCL (HR, 0.89; 95%CI, 0.54-1.45). The HIV status was not an independent prognostic factor in PEL, PBL, or HHV8+DLBCL. Advanced stage was prognostic for PBL (p=.0002), but not for ALK+LBCL (p=.96), or HHV8+DLBCL (p=.28). In PEL and PBL survival significantly differed according to primary site. Novel therapeutic approaches are urgently needed for these rare diseases.