[1438] Clinicopathologic Features of Anaplastic Large Cell Lymphoma (ALCL), ALK- and CD30+ Peripheral T-Cell Lymphoma, Not Otherwise Specified (PTCL, nos): A Study from the North American T-Cell Lymphoma Consortium

William R Macon, Matthew J Maurer, Jonathan Said, Scott J Rodig, Randy D Gascoyne, David M Dorfman, Sarah Ondrejka, Ahmet Dogan, Eric D Hsi. Mayo Clinic, Rochester, MN; UCLA, Los Angeles, CA; Brigham and Women's Hospital, Boston, MA; BCCA, Vancouver, Canada; Cleveland Clinic, Cleveland, OH

Background: The clinicopathologic features of ALCL, ALK- have not been well-characterized, particularly in relation to PTCL, nos. We identified cases of ALCL, ALK- and CD30+ PTCL, nos in order to compare clinicopathologic features that might better define them.
Design: Cases of ALCL and PTCL, nos were collected as part of a collaborative study. Cases were reviewed independently by 3 hematopathologists with an immunophenotypic panel consisting of stains for CD3, CD5, CD10, CD20, CD21, CD30, CD45, PAX5, CD2, CD4, CD7, CD8, CD23, PD1, CD56, ALK, TIA1, TCRδ, TCRβF1, and EBER in situ hybridization. Diagnosis was assigned by unanimous agreement or consensus, in case of disagreement. Phenotype comparison between ALK- ALCL and other groups was done via chi-squares tests and using Kaplan-Meier and log-rank tests for overall survival (OS).
Results: 159 total cases of ALCL and PTCL, nos were identified. Clinical and phenotypic features are contrasted in Table 1.

Table 1: Clinical and Pathologic Features
 ALCL, ALK- (N=37)CD30+ PTCL, nos (N=21)PALCL, ALK+ (N=27)PCD30- PTCL, nos (N=74)P
Median Age63670.30310.0003640.40
CD426 (70%)13 (62%)0.5112 (46%)0.05442 (59%)0.26
CD85 (14%)2 (10%)0.702 (8%)0.4715 (21%)0.35
CD100 (0%)1 (5%)0.190 (0%)1.001 (1%)0.48
TCRβ7 (20%)12 (57%)0.00456 (23%)0.7757 (79%)<0.0001
TCRδ0 (0%)3 (15%)0.0170 (0%)1.005 (7%)0.10
CD561 (3%)1 (5%)0.681 (4%)0.7810 (14%)0.072
TIA19 (25%)5 (24%)0.9214 (54%)0.02032 (44%)0.050
PD11 (3%)8 (38%)0.00030 (0%)0.4220 (29%)0.0011
Loss of CD2, CD5, or CD735 (95%)17 (67%)0.004826 (96%)0.7544 (60%)0.0001
Overall Survival16.83.30.36Unreached0.238.10.21

Conclusions: We have identified a series of CD30+ PTCL, nos and ALCL, ALK- cases through a process of central review with a standard set of immunophenotypic markers. Compared to ALK- ALCL, CD30+ PTCL, nos were more likely to express T-cell receptor molecules and the Tfh marker PD1. They were less likely to lose pan-T cell markers. Similar differences were seen between ALK- ALCL and CD30- PTCL, nos. ALK+ ALCL was more likely to express the cytotoxic marker TIA1 compared to ALK- ALCL. OS of ALK- ALCL was intermediate between ALK+ ALCL and CD30+ PTCL nos, p=0.18. The phenotypic differences likely reflect underlying biologic differences between ALK- ALCL and CD30+ and CD30- PTCL, nos and support current classification of ALK- ALCL as a distinct entity.
Category: Hematopathology

Tuesday, March 5, 2013 1:30 PM

Proffered Papers: Section G, Tuesday Afternoon


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