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Blood, 15 June 2008, Vol. 111, No. 12, pp. 5496-5504.
Prepublished online as a Blood First Edition Paper on April 2, 2008; DOI 10.1182/blood-2008-01-134270.
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Submitted January 28, 2008
Accepted March 18, 2008
ALK-negative anaplastic large-cell lymphoma (ALCL) is clinically and immunophenotypically different from both ALK-positive ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project
Kerry J Savage*, Nancy Lee Harris, Julie M Vose, Fred Ullrich, Elaine S Jaffe, Joseph M Connors, Lisa Rimsza, Stefano A Pileri, Mukesh Chhanabhai, Randy D Gascoyne, James O Armitage, and Dennis D Weisenburger
Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, British Columbia, Canada
Department of Pathology, Massachusetts General Hospital, Boston, MA, United States
Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, United States
Department of Preventive and Societal Medicine, University of Nebraska Medical Center, Omaha, NE, United States
Department of Hematopathology, National Cancer Institute, Bethesda, MD, United States
Department of Medical Oncology, British Columbia Cancer Agency, Vancouver, BC, Canada
Department of Pathology, University of Arizona, Tucson, Arizona, United States
Department of Pathology, Bologna University School of Medicine, Bologna, Italy
Department of Pathology, British Columbia Cancer Agency, Vancouver, BC, Canada
Department of Internal Medicine, Univeristy of Nebraska Medical Center, Omaha, NE, United States
Department of Pathology, Univeristy of Nebraska Medical Center, Omaha, NE, United States
* Corresponding author; email: ksavage{at}bccancer.bc.ca.
The International Peripheral T-cell Lymphoma Project is a large collaborative effort designed to gain better understanding of peripheral T- or NK/T-cell lymphomas (PTCLs) Twenty-two institutions in North America, Europe and Asia submitted clinical and pathologic information on PTCLs diagnosed and treated at their respective centers. Of the 1314 eligible cases, 181 had anaplastic large cell lymphoma (ALCL) (13.8%) on consensus review: 159 systemic ALCL (12.1%) and 22 primary cutaneous ALCL (1.7%). ALK-positive ALCL patients had a superior outcome compared to those with ALK-negative ALCL (5 year (y) failure-free survival (FFS) 60% vs 36%, p=.015; 5 y overall survival (OS) 70% vs 49%, p=.016). However, contrary to prior reports, the 5 y FFS (36% vs 20% p=.012) and OS (49% vs 32% p=.032) was superior for ALK-negative ALCL compared to PTCL, not otherwise specified (PTCL-NOS). Primary cutaneous ALCL patients had a very favorable 5 y OS (90%), but with a propensity to relapse (5 y FFS 55%). In summary, ALK-negative ALCL should continue to be separated from both ALK-positive ALCL and PTCL-NOS. Although the prognosis of ALK-negative ALCL appears to be better than PTCL-NOS, it is still unsatisfactory and better therapies are needed. Primary cutaneous ALCL is associated with an indolent course.
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