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Primary Anaplastic Large-Cell Lymphoma in Adults: Clinical Presentation, Immunophenotype, and Outcome

Hervé Tilly, Philippe Gaulard, Eric Lepage, Charles Dumontet, Jacques Diebold, Isabelle Plantier, Françoise Berger, Michel Symann, Tony Petrella, Pierre Lederlin, and Josette Brière for the Groupe d'Etudes des Lymphomes de l'Adulte

From the Centre Henri Becquerel, Rouen; the Hôpital Henri Mondor, Créteil; the Centre Hospitalier Lyon-Sud, Pierre Bénite; the Hôtel-Dieu, Paris; the Centre Hospitalier Régional, Lille; the Hôpital Edouard Herriot, Lyon; the Centre Hospitalier Universitaire; Dijon, the Centre Hospitalier Régional, Vandoeuvre les Nancy; the Hôpital Laënnec, Paris, France; and the Hôpital Saint-Luc, Brussels, Belgium.

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.

Blood, Vol. 90 No. 9 (November 1), 1997: pp. 3727-3734
© 1997 by The American Society of Hematology.


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