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Chapter 85 T-Cell Lymphomas 1349

Anaplastic large cell lymphoma, ALK+
Anaplastic large cell lymphoma, ALK–
All natural killer/T-cell lymphomas
Peripheral T-cell lymphoma, not otherwise specified
100 Angioimmunoblastic lymphoma
90 Adult T-cell leukemia/lymphoma
80
Overall survival (%) 60
70
50
40
30
20
10 p < .001
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
A Time (years)

Primary cutaneous ALCL
Subcutaneous panniculitis-like T-cell lymphoma
Enteropathy-type T-cell lymphoma
Hepatosplenic T-cell lymphoma

100
90
80
Overall survival (%) 60
70
50
40
30
20
10 p < .001
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13
B Time (years)

100 Nasal natural killer/T-cell lymphoma
90 Natural killer/T-cell lymphoma, nasal type
Aggressive or unclassifiable natural killer/T-cell lymphoma
80
Overall survival (%) 60
70
50
40
30
20
10 p < .001
0
0 1 2 3 4 5 6 7 8 9 10 11 12
C Time (years)
Fig. 85.6 (A) Overall survival of patients with the common subtypes of peripheral T-cell lymphoma (PTCL).
(B) Overall survival of patients with less common subtypes of PTCL. (C) Overall survival of patients with
natural killer/T-cell lymphoma. (Data from Vose J, Armitage J, Weisenburger D: International peripheral T-cell and
natural killer/T-cell lymphoma study: Pathology findings and clinical outcomes. J Clin Oncol 26:4124, 2008.)

outcomes for PTCL patients are substantially inferior to those for subtypes including PTCL-NOS, AITL, ALK+ and ALK− T-cell
their B-cell lymphoma counterparts. Lymphomas derived from the lymphomas. The median time from diagnosis to relapse or progres-
T-cell lineage have been shown to be an independent negative prog- sion of disease after primary therapy was 6.7 months. The median
nostic factor. The adverse prognosis of patients with relapsed or overall survival (OS) and second progression-free survival were only
refractory PTCL has been highlighted in a recent experience from 5.5 and 3.1 months, respectively, which was only marginally improved
the British Columbia Cancer Agency (BCCA). In this series, the if those patients went on to receive subsequent chemotherapy. This
BCCA identified 153 patients who relapsed or progressed after experience underscores the poor performance of conventional treat-
primary therapy. They identified 153 patients with a variety of PTCL ment regimens in the treatment of the disease, highlights the