title: Primary anaplastic large-cell lymphoma in adults: clinical presentation, immunophenotype, and outcome.
abstract: 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, but 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.
authors.affiliation: Centre Henri Becquerel, Rouen, France.
authors.forename: H
authors.initials: H
authors.lastname: Tilly
authors.forename: P
authors.initials: P
authors.lastname: Gaulard
authors.forename: E
authors.initials: E
authors.lastname: Lepage
authors.forename: C
authors.initials: C
authors.lastname: Dumontet
authors.forename: J
authors.initials: J
authors.lastname: Diebold
authors.forename: I
authors.initials: I
authors.lastname: Plantier
authors.forename: F
authors.initials: F
authors.lastname: Berger
authors.forename: M
authors.initials: M
authors.lastname: Symann
authors.forename: T
authors.initials: T
authors.lastname: Petrella
authors.forename: P
authors.initials: P
authors.lastname: Lederlin
authors.forename: J
authors.initials: J
authors.lastname: Brière
mesh_terms: D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D017728:Lymphoma, Large-Cell, Anaplastic / Q000276:immunology / Q000401:mortality / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome