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.
Keywords: ALK-positive large B-cell lymphoma; Diffuse large B-cell lymphoma; epidemiology; multicentric Castleman disease; plasmablastic lymphoma; primary effusion lymphoma.