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1632 Part XI: Malignant Lymphoid Diseases Chapter 98: Diffuse Large B-Cell Lymphoma and Related Diseases 1633

Overall survival Death rate per year Figure 98–1. (Left panels) Kaplan-Meier survival curves for the
94
100 30 four risk groups. (Right panels) Death rates during the study
period. Only 2031 of the 3273 patients had sufficient relevant
20 All information for classification according to the international
50 patients index. H, high risk; HI, high-intermediate risk; L, low risk; LI,
10 (n = 3273) low-intermediate risk.

100 30 H
20 HI Training
L
50 HI 10 LI sample
LI
(n = 1385)
% of patients 100 H 30 L H

L
sample
50 LI 20 HI Validation
HI 10 LI (n = 646)
H
L
100 30 H
20 Patients with
50 L HI complete data
LI
LI
HI 10 (n = 2031)
H L
0
0 0 2 4 6 8 10 0 2 4 6 8 10
Year

by fluorescence in situ hybridization (FISH) have been shown to have POSITRON EMISSION TOMOGRAPHY
an inferior 5-year survival of 33 percent when treated with standard
R-CHOP therapy, compared to 72 percent survival for those without Fluorine-18-fluorodeoxyglucose-positron emission tomography (FDG-
MYC rearrangements. Concurrent translocation of BCL2 or BCL6 PET) is used for initial staging and at the end of treatment (to assess
100
is seen in a subset of cases. These “double-hit” lymphomas are often remission status) in patients with DLBCL. FDG-PET is superior to
classified morphologically as “B-cell lymphoma, unclassifiable, with computed tomography imaging in detecting nodal and extranodal sites
features intermediate between DLBCL and Burkitt lymphoma” using of aggressive lymphoma, with the potential to alter stage, prognosis, and
103
the 2008 WHO classification of lymphomas and have a very bad prog- selection of therapy. In addition, FDG-PET performed at the end of
nosis. MYC protein overexpression by IHC is also associated with an therapy is highly informative. A negative PET predicts a high proba-
2
inferior prognosis, particularly when accompanied by BCL2 protein bility of disease control, and is required to designate a CR by modern
33,104
overexpression. 101,102 response criteria. Other uses of PET in DLBCL are investigational
or not routinely recommended.
TABLE 98–6. Outcome According to Risk Group Defined by the International Prognostic Index 93
Complete
International Index No. of Risk Factors Response Rate (%) Relapse-Free Survival (%) Survival (%)
INTERNATIONAL PROGNOSTIC INDEX, ALL PATIENTS
2-Year 5-Year 2-Year 5-Year
Low 0 or 1 87 79 70 84 73
Low-intermediate 2 67 66 50 66 51
High-intermediate 3 55 59 49 54 43
High 4 or 5 44 58 40 34 26
AGE-ADJUSTED INTERNATIONAL INDEX, PATIENTS <61 YEARS OF AGE
2-Year 5-Year 2-Year 5-Year
Low 0 92 88 86 90 83
Low-intermediate 1 78 74 66 79 69
High-intermediate 2 57 62 53 59 46
High 3 46 61 58 37 32

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