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Advances in Hematology
Volume 2010, Article ID 345053, 10 pages
http://dx.doi.org/10.1155/2010/345053
Review Article

Pathobiology of Anaplastic Large Cell Lymphoma

1Hematopathology Section, Department of Hematology and Oncological Sciences “L. and A. Seràgnoli”, S. Orsola-Malpighi Hospital, University of Bologna, 40138 Bologna, Italy
2Molecular Pathology Laboratory, Haematopathology Section, Department of Hematology and Oncological Sciences “L. and A. Seràgnoli”, S. Orsola-Malpighi Hospital, University of Bologna, Pavillon 8, Via Massarenti 9, 40138 Bologna, Italy
3Center for Experimental Research and Medical Studies (CERMS), University of Torino, 10126 Torino, Italy
4Department of Haematology, St. George Hospital, Clinical Services Building, Kogarah NSW 2217, Australia

Received 31 August 2010; Revised 24 November 2010; Accepted 12 December 2010

Academic Editor: Shaji Kumar

Copyright © 2010 Pier Paolo Piccaluga et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The authors revise the concept of anaplastic large cell lymphoma (ALCL) in the light of the recently updated WHO classification of Tumors of Hematopoietic and Lymphoid Tissues both on biological and clinical grounds. The main histological findings are illustrated with special reference to the cytological spectrum that is indeed characteristic of the tumor. The phenotype is reported in detail: the expression of the ALK protein as well as the chromosomal abnormalities is discussed with their potential pathogenetic implications. The clinical features of ALCL are presented by underlining the difference in terms of response to therapy and survival between the ALK-positive and ALK-negative forms. Finally, the biological rationale for potential innovative targeted therapies is presented.