Nodal cytotoxic lymphoma spectrum - A clinicopathologic study of 66 patients

The expression of cytotoxic granule-associated proteins has been reported i n some T-cell or natural killer (NK)-cell lymphomas of mostly extranodal or igin, but rarely of nodal origin except for anaplastic large cell lymphoma (ALCL) and Hodgkin's disease (HD). This study analyzed 66 nodal lymphomas e xpressing T-cell intracellular antigen-1 (TIA-1) and/or granzyme B to chara cterize the clinicopathologic spectrum of these neoplasms. Four main groups could be delineated. The first group consisted of p80/anaplastic lymphoma kinase (ALK)-positive ALCL (n = 35). The patients were 2 to 62 years of age (median age, 16 years), and the lymphomas pursued a relatively indolent cl inical course. The tumors were phenotypically of either T- or null-cell typ e with constant expression of CD30, epithelial membrane antigen (EMA), and p80/ALK, bur not CD15 or BCL2. None harbored Epstein-Barr virus (EBV). The second group consisted of peripheral T/NK-cell lymphoma, the nodal high-gra de cytotoxic type (n = 13). The patients were 29 to 72 years in age (median age, 55 years), and the tumors pursued an aggressive clinical course. The tumors often showed pleomorphic, anaplastic, or centroblastoid morphology, and were featured by either EBV association or CD56 expression. The third g roup consisted of peripheral T-cell lymphoma, of the nodal low-grade cytoto xic type(n = 8). The patients, three men and five women, were 31 to 75 year s old (median age, 61 years). Notably. six of them exhibited lymphoepitheli oid (Lennert's) lymphoma. The fourth group consisted of cytotoxic Hodgkin's -like ALCL/HD (n = 10), included seven cases of Hodgkin's-like ALCL and thr ee cases of HD, and was characterized by the presence of Reed-Sternberg cel ls and often the CD15(+) phenotype. The patients were all men except for on e woman, and they ranged in age from 24 to 84 years (median age, 62 years). The link among these four groups was reinforced by the presence of a highl y characteristic large cell with horseshoelike or reniform nuclei-the frequ ent expression of CD30 and EMA-and the often lack of T-cell receptor-ap. In this series, the expression of p80/ALK and CD56 was also associated with f avorable and poor prognoses respectively (p < 0.001, log-rank test).