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Nodal cytotoxic molecule ( CM )‐positive peripheral T‐cell lymphoma ( CTL ) has recently been recognized as a clinicopathologically distinct disease. To further characterize this disease, here we compared 58 patients with Epstein‐Barr virus ( EBV )‐negative  CTL  to 48 patients with  EBV ‐positive  CTL . The two groups did not differ in histopathology, T‐cell receptor ( TCR ) expression or rearrangement incidences, or survival curves. However, patients with  EBV ‐negative  CTL  less frequently showed hepatic involvement ( P  = .007), B symptoms ( P  = .020), hemophagocytosis ( P  = .024), and detectable  CD 4 ( P  = .002) and  CD 5 ( P  = .009). Univariate and multivariate analyses identified three factors that independently predicted favorable survival, onset age <60 years ( P  = .002),  CD 5 expression ( P  = .002), and mixed morphology ( P  = .013),  TCR αβ was not an independent predictor ( P  = .30), but was strongly linked with long survivorship among patients younger than 60 years old. A prognostic model incorporating these factors worked well for prognostic delineation, independently of the International Prognostic Index ( P  = .007 vs  P  = .082) and Prognostic Index for  PTCL  ( P  = .020 vs  P  = .15). Moreover, this constellation of findings indicated two nodal indolent diseases:  CD 5 +  TCR αβ (n = 13), and  CD 5 +   NK ‐cell type lacking  TCR  expression or clonal   TCR  γ rearrangement (n = 4). The survival curves for these two groups were significantly superior to others (n = 29,  P  < .001). These diseases appear to be unique in their indolent clinical behavior, and should be managed differently from other diseases.

Reappraisal of nodal Epstein‐Barr Virus‐negative cytotoxic T‐cell lymphoma: Identification of indolent CD 5 + diseases