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Chapter 85 T-Cell Lymphomas 1351

100
p < .001 International Prognostic Index
90
80 0/1
2
Overall survival (%) 60 4/5
70
3
50
40
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
A Time (years)

100
90
p < .001 International Prognostic Index
Failure-free survival (%) 60 2
80
0/1
70
3
50
4/5
40
30
20
10
0
0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18
B Time (years)
Fig. 85.8 OVERALL SURVIVAL (A) AND FAILURE-FREE SURVIVAL (B) OF 315 PATIENTS WITH
PERIPHERAL T-CELL LYMPHOMA NOT OTHERWISE SPECIFIED ACCORDING TO THE INTER-
NATIONAL PROGNOSTIC INDEX. (Data from Weisenburger DD, Savage KJ, Harris NL, et al: Peripheral T-cell
lymphoma, not otherwise specified: A report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood
117:3402, 2011.)

Probably the most important prognostic factor for any subtype of lymphoma established that CHOP exhibited the same efficacy and
PTCL is the presence or absence of ALK in ALCL. The OS of less toxicity than the other regimens. This landmark trial was based
ALK-positive ALCL is substantially better than that seen for ALK- on the use of a histopathologic classification system when immuno-
negative ALCL (71% ± 6% vs. 15% ± 11%, respectively). However, phenotyping was not yet routinely applied to all cases. Unfortunately,
within the good prognostic category of ALK-positive ALCL, survival there are no large randomized prospective studies that compare the
was 94% ± 5% for the low/low-intermediate risk group (age-adjusted benefit of anthracycline-based therapies to other combination regi-
IPI 0–1) and 41% ± 12% for the high/high-intermediate risk group mens in PTCL.
(age-adjusted IPI ≥2). Multivariate analysis identified ALK expression Conventional front-line chemotherapy such as CHOP has led to
and the IPI as independent variables that were able to predict survival disappointing results in patients with T-cell lymphomas. Analysis of
among T/null primary, systemic ALCL. the data as a function of the primary PTCL subtypes revealed that
More recently, recognizing the biologic and clinical heterogeneity only ALCL with the t(2;5) translocation (NPM-ALK fusion protein)
of PTCL, investigators have begun to develop subtype-specific had an equivalent or superior prognosis compared with patients with
prognostic models. Although potentially interesting, these models DLBCL. The LNH87 study conducted by the Groupe d’Etudes des
still need to be validated in large studies. Lymphomes de l’Adulte showed that the use of anthracycline-
containing chemotherapy regimens in T- and B-cell lymphomas were
Therapy able to induce the complete remission (CR) in 54% versus 63% of
the patients, with an OS of 41% and 53%, and an event-free survival
(EFS) of 33% and 42%, respectively. The same group published the
Standard of Care results of the LNH84 study, in which they employed a dose-intense
consolidation. Again, although there was no difference in the response
CHOP-Like Therapy in Mature T-Cell Lymphomas rate (RR), patients with T-cell lymphomas relapsed more frequently
Treatment approaches employed for patients with PTCL have largely and earlier compared with patients with B-cell lymphoma. More
mirrored the strategies employed for the treatment of DLBCL. recently the CHOEP regimen (cyclophosphamide, hydroxydauno-
Consequently, CHOP (cyclophosphamide, hydroxydaunomycin, mycin, vincristine [Oncovin], etoposide, and prednisone) was retro-
vincristine [Oncovin], and prednisone) has emerged as the “standard spectively studied by the German High-Grade Non-Hodgkin
of care” despite disappointing outcomes. The milestone Southwest Lymphoma Study Group in 343 patients with T-cell lymphoma,
Oncology Group (SWOG) trial that compared CHOP with the including PTCL-NOS, ALCL, and AITL, who had been included in
second- and third-generation dose-intensive regimens in aggressive seven German high-grade phase II or III aggressive NHL treatment