Page 255 - Haematologica March 2020

Outcomes of Richter transformation
A
B
C
D
Figure 2. Overall survival (OS) after Richter transformation (RT) diagnosis by clinical and molecular factors. (A) OS by age at RT diagnosis. (B) OS by lactate dehy- drogenase (LDH) at RT diagnosis. (C) OS by TP53 disruption status. (D) OS by Myc and Bcl-2 double expression status.
Table 2. First-line treatment approaches for Richter transformation (RT) in patients with chronic lymphocytic leukemia (CLL). Prior CLL treatment
R-CHOP-like regimen1
Platinum or high dose cytarabine containing chemotherapy2 Other chemotherapy3
Novel agents4
Palliative therapy5
Missing
Total
All patients (%)
114 (65.5%)
12 (6.9%) 21 (12.1%) 19 (10.9%) 8 (4.6%) 30
204
None (%)
49 (81.7%)
1 (1.7%)
9 (15.0%)
0 (0.0%)
1 (1.7%)
9
69
Chemoimmunotherapy only (%)
60 (66.7%)
10 (11.1%)
10 (11.1%)
4 (4.4%)
6 (6.7%)
18
108
At least one novel agent (%)
5 (20.8%)
1 (4.2%)
2 (8.3%)
15 (62.5%)
1 (4.2%)
3
27
1Predomidantly R-CHOP, small numbers of CHOP, MR-CHOP, lenalidomide-R-CHOP. 2R-ICE, (R-)DHAP, (R-)ESHAP, GDP, R-Hyper-CVAD, CODOX-M-IVAC, OFAR. 3(R-)EPOCH, ProMACE-CytaBOM,(R-)CEPP, infusionalCDE,R-CVP,R-bendamustine,R-gemcitabine/prednisone, high-doseMTX-based.4Ibrutinib,venetoclax,pembrolizumab,everolimus, CD19 antibody. 5Rituxiamab,obinutuzumab,corticosteroids,radiation therapy, alone or in combination.
cytarabine containing chemotherapy (n=12) had a median OS of 14.6 months (P=0.82 vs. R-CHOP-like). Patients treated with other chemotherapy (n=21) had a median OS of 12.8 months (P=0.66 vs. R-CHOP-like). The 19 patients who received novel agents as first-line RT treatment had a median OS of 10.9 months (P=0.12 vs. R-CHOP-like). The median OS for patients receiving palliative therapy (n=8) was 6.1 months (P=0.01 vs. R-CHOP-like).
After achieving PR or better, 24 (11.8%) patients under- went SCT: 20 autologous and 4 allogenic. Details of the clinical characteristics of the 24 patients who underwent SCT are shown in Online Supplementary Table S2. The median age at RT diagnosis was 62 years (range 41-73). Ten patients did not receive any prior CLL treatment. Nineteen patients achieved a PR or better with 1-2 lines of
treatment before proceeding to SCT. The median time from RT diagnosis to SCT was 6.8 months (range 3.3- 42.3). None of the four allogeneic SCT patients died although the post-SCT follow up was still relatively short for three of them (Figure 3A). Thirteen of the 20 autolo- gous SCT patients had a post-SCT survival greater than two years (Figure 3A). Overall, the 24 SCT patients had a median post-SCT survival of 55.4 months (Figure 3B).
Discussion
To the best of our knowledge, this is the largest series of CLL patients with biopsy-proven DLBCL-type RT with a long follow up. Our results showed that patients with
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