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1352 Part VII Hematologic Malignancies

PERIPHERAL T-CELL LYMPHOMA NOT OTHERWISE SPECIFIED
Prognostic Index for PTCL-NOS
100
0
90 1
80 2 3/4
Overall survival (%) 60
70
50
40
30
20
10
0
A 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

Time (years)
PERIPHERAL T-CELL LYMPHOMA NOT OTHERWISE SPECIFIED
100
90
Prognostic Index for PTCL-NOS
80
Failure-free survival (%) 60 1 2 3/4
0
70
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
B Time (years)
Fig. 85.9 OVERALL SURVIVAL (A) AND FAILURE-FREE SURVIVAL (B) OF 315 PATIENTS WITH
PERIPHERAL T-CELL LYMPHOMA NOT OTHERWISE SPECIFIED ACCORDING TO THE PROG-
NOSTIC INDEX. PTCL-NOS, Peripheral T-cell lymphoma not otherwise specified. (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.)
studies. The main purpose was to determine whether the addition of close to 80%. Patients who experience relapse of their disease gener-
etoposide or shortening of the interval of chemotherapy from 3 weeks ally exhibit the same poor prognosis as patients with other subtypes
to 2 weeks affected survival. In older adult patients, neither shortening of PTCL. Patients with ALK-negative ALCL carry the same poor
the interval nor the addition of etoposide improved the EFS and OS. prognosis as other subtypes of mature T-cell lymphoma, and may be
A recent literature review and meta-analysis reported a CR rate of suitable candidates for clinical trials in both the upfront and relapsed
52% achieved with CHOP/CHOP-like regimens (excluding ALK- settings.
positive ALCL), with an estimated 5-year OS of only 35% in PTCL.
This is in contrast to the 65% or better long-term survival frequently Nasal NKTCL: Combined-Modality Versus Single-
reported in DLBCL. Select studies reporting on the activity of various Modality Approaches
combination regimens in PTCL are presented in Table 85.2. Radiotherapy is considered the most active treatment for early-stage
nasal NKTCL. For patients with limited stage IE or stage IE disease
Anaplastic Large-Cell Lymphoma without any adverse factors, radiotherapy alone should be pursued
ALCL is a unique subtype of an aggressive mature T-cell lymphoma with curative intent. Radiation produces complete RR of up to 70%;
that carries a highly favorable prognosis, especially for patients with however, approximately 50% of patients who receive radiation alone
who carry the ALK translocation [the NPM-ALK; t(2:5)] with IPI 1 will relapse, with about 25% of patients experiencing a systemic
or 2 disease. Virtually all cases of ALCL express CD30, and about relapse. Hence, the addition of chemotherapy is intended to reduce
half of the patients with this disease harbor the t(2;5)(p21;q35) the risk or recurrence, and is essential for advanced stages of nasal
translocation. This chromosomal aberration leads to fusion of the and extranasal NKTCL. For patients with extensive stage I and II
NPM gene with the ALK tyrosine kinase, leading to its constitutive disease, radiotherapy followed by consolidation with chemotherapy
activation. The presence of the ALK translocation confers a highly is commonly used. Because of the intrinsic drug resistance frequently
favorable prognosis, and determination of ALK’s status by FISH is seen in this disease, chemotherapy is not recommended as primary
imperative in all cases of ALCL, especially those in which the treatment in early-stage nasal NKTCL. Extranodal NKTCL is gener-
differential diagnosis also includes PTCL-NOS. The National Com- ally refractory to CHOP-based chemotherapy and is often associated
prehensive Cancer Network guidelines recommend standard CHOP- with the expression of multidrug resistance (MDR) genes.
based chemotherapy for these patients, sometimes combined with Despite the benefit of radiotherapy in this disease, a uniformly
radiation for bulky disease, which typically produces remission rates accepted standard of care has yet to be identified. Recently a phase