Primary anaplastic large-cell lymphoma in adults: Clinical presentation, immunophenotype, and outcome

Anaplastic, CD30(+), large-cell lymphoma is now a well-recognized pathologic entity that accounts for 2% to 8% of all lymphomas, Recent progress has been made in the understanding of certain biologic features found in anaplastic large-cell lymphoma, but information about its clinical 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 lymphoma (ALCL) on the basis of morphology and CD30 expression. We compared initial presentation, immunophenotype, and clinical outcome of these cases with those of the 1,695 nonanaplastic diffuse large-cell lymphomas (non-ALCL) included in the same trial. Patients with ALCL were more likely to be male (P = .018) and were younger (P <.0001) than those with non-ALCL. B symptoms were more frequent in ALCL (P = .006). Skin (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-free survival (P = .006), and overall survival (P = .0004) were better for 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) or overall survival (P = .83). ALCL in adults is a clinicopathologic entity which, independent of its phenotypic characteristics, has a better outcome than other diffuse large-cell lymphomas. (C) 1997 by The American Society of Hematology.