PRIMARY ANAPLASTIC LARGE-CELL LYMPHOMA IN ADULTS - CLINICAL PRESENTATION, IMMUNOPHENOTYPE, AND OUTCOME

Anaplastic, CD30(+), large-cell lymphoma is now a well-recognized path ologic entity that accounts for 2% to 8% of all lymphomas, Recent prog ress has been made in the understanding of certain biologic features f ound in anaplastic large-cell lymphoma, but information about its clin ical behavior, in comparison to other large-cell lymphomas, is limited , The pathologic review of a large multicenter study of the treatment of aggressive lymphoma identified 146 cases of anaplastic large-cell l ymphoma (ALCL) on the basis of morphology and CD30 expression. We comp ared initial presentation, immunophenotype, and clinical outcome of th ese cases with those of the 1,695 nonanaplastic diffuse large-cell lym phomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P <.0001) than tho se with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Sk in (P <.0001) and lung (P <.05) involvement was also more frequent in ALCL, hut frequency of bone marrow involvement was identical (P =.5). Tumor cell phenotype was B in 56 cases (38%), T in 49 cases (34%), and null in 33 cases (22%). Response to chemotherapy (P = .001), event-fr ee survival (P = .006), and overall survival (P = .0004) were better f or ALCL than for non-ALCL. Multivariate analyses identified anaplastic character as an independent factor that predicted a longer survival, Tumor cell phenotype did not influence event-free survival (P = .72) o r overall survival (P = .83). ALCL in adults is a clinicopathologic en tity which, independent of its phenotypic characteristics, has a bette r outcome than other diffuse large-cell lymphomas. (C) 1997 by The Ame rican Society of Hematology.