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E.D. McPhail et al.
(P<0.001). All 13 MYC/BCL6 cases were de novo diag- noses, whereas 38% of DH-BCL2/THL cases were either transformed (n=22) or relapsed (n=11) (P=0.03). No asso- ciation was observed between morphological features and BCL2 rearrangement status (P=0.16).
Therapy, clinical characteristics, and outcome
The therapeutic regimens were R-CHOP (n=36); dose- adjusted EPOCH-R (n=17); R-CODOX-M/IVAC (n=17); R-hyper-CVAD, methotrexate, and cytarabine (n=6); plat- inum-based salvage (n=10); and non-anthracycline-based treatment (n=14). Survival was analyzed for 70 patients receiving anthracycline-based curative-intent therapy at diagnosis of DH/THL (Table 1). The median (interquartile range) age of these patients was 61 (29-82) years; 41 (59%) were males. Fifty-nine patients had de novo DH/THL and 11 had transformation of a previously diag- nosed low-grade lymphoma. For treatment, 32 patients
received R-CHOP; 17, dose-adjusted EPOCH-R; 15, R- CODOX-M/IVAC; and 6, R-hyper-CVAD with methotrexate and cytarabine. Twelve patients (17%) received consolidation with autologous stem cell trans- plantation. At a median (range) follow-up of 21 (1-87) months, EFS12 was 46% (Figure 3A) and median OS was 22 months (95% CI: 13 months - unreached) (Figure 3B). At a 5-year follow-up, the OS rate was 49% (95% CI: 37%-64%).
Patients with DH/THL at transformation of previously diagnosed low-grade lymphoma (n=11) had poor out- comes [median OS, 10.8 months; hazard ratio (HR), 2.99; 95% CI: 1.33-6.71; EFS12, 10%] - inferior to the out- comes of patients with de novo DH/THL (median OS, 22 months; P=0.008; EFS12, 52%) (Figure 4A). However, seven of the 11 patients had received prior immunochemotherapy for their low-grade lymphoma. Patients with high-grade morphological features on a con-
A
AB
Figure 1. Comparison of high- grade and large-cell cytologic characteristics. (A) Double- hit/triple-hit lymphoma (DH/THL) with a high-grade morphological pattern (hematoxylin-eosin, origi- nal magnification ×400). (B) DH/THL with large-cell morpholog- ical characteristics (hematoxylin- eosin, original magnification ×400).
Figure 2. MYC rearrangement partners and BCL2 and BCL6 rearrangement status. (A) MYC rearrangement partners in dou- ble-hit/triple-hit lymphoma (DH/THL) by interphase dual- fusion fluorescence in situ hybridization (FISH). Cases with an IG/MYC rearrangement (IGH/MYC, IGK/MYC, or IGL/MYC) are contiguous. (B) BCL2 and BCL6 rearrangement status in DH/THL by interphase FISH. All cases had a MYC rearrangement. BCL2-rearranged cases are con- tiguous whereas MYC/BCL6 cases are not contiguous.
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