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Blood, 1 December 2000, Vol. 96, No. 12, pp. 3681-3695

REVIEW ARTICLE

CD30+ anaplastic large cell lymphoma: a review of its histopathologic, genetic, and clinical features

Harald Stein, Hans-Dieter Foss, Horst Dürkop, Theresa Marafioti, Georges Delsol, Karen Pulford, Stefano Pileri, and Brunangelo Falini

From the Institute of Pathology and Consultation and Reference Center for Lymph Node Pathology and Haematopathology, University Hospital Benjamin Franklin, Free University, Berlin, Germany; Laboratoire d' Anatomie et Cytologie Pathologiques, Hôpital Purpan, Toulouse, France; Nuffield Department of Clinical Laboratory Sciences, LRF Immunodiagnostics Unit, John Radcliffe Hospital, University of Oxford, Oxford, England; Servizio di Anatomia Patologica---Sezione di Emolinfopatologia, Università degli Studi di Bologna, Italy; and Dipartimento di Medicina Clinica e Sperimentale, Sezione di Ematologia e Immunologia Clinica, Policlinico Monteluce, Università degli Studi di Perugia, Italy.

Anaplastic large cell lymphoma (ALCL) represents a generally recognized group of large cell lymphomas. Defining features consist of a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 and a characteristic growth pattern. With the use of molecular and clinical criteria, 3 entities of ALCL have been identified: primary systemic anaplastic lymphoma kinase (ALK)+ ALCL, primary systemic ALK- ALCL, and primary cutaneous ALCL. ALK expression is caused by chromosomal translocations, most commonly t(2;5). ALK+ ALCL predominantly affects young male patients and, if treated with chemotherapy, has a favorable prognosis. It shows a broad morphologic spectrum, with the "common type," the small cell variant, and the lymphohistiocytic variant being most commonly observed. The knowledge of the existence of these variants is essential in establishing a correct diagnosis. ALK- ALCL occurs in older patients, affecting both genders equally and having an unfavorable prognosis. The morphology and the immunophenotype of primary cutaneous ALCL show an overlap with that of lymphomatoid papulosis. Both diseases have an excellent prognosis, and secondary systemic dissemination is only rarely observed. The described ALCL entities usually derive from cytotoxic T cells. In contrast, large B-cell lymphomas with anaplastic morphology are believed to represent not a separate entity but a morphologic variant of diffuse large B-cell lymphoma. Malignant lymphomas with morphologic features of both Hodgkin disease and ALCL have formerly been classified as Hodgkin-like ALCL . Recent immunohistologic studies, however, suggest that ALCLs Hodgkin-like represent either cases of tumor cell-rich classic Hodgkin disease or (less commonly) ALK+ ALCL or ALK- ALCL.

© 2000 by The American Society of Hematology.
 

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