Table 1. . Published studies reporting outcomes in Richter's syndrome diffuse large B-cell lymphoma specific cohorts.
| Study agents | Year reported | Phase | Studied patients | Total no. enrolled (no. RS) | ORR (RS specific) | CR (RS specific) | PFS in months (median) | OS in months (median) |
|---|---|---|---|---|---|---|---|---|
| HyperCVXD 59 | 2001 | II | RS | 29 (29) | 41% | 38% | NR | 10 |
| FACPGM 60 | 2002 | II | RS, PLL, NHL | 22 (15) | 5% | 5% | NR | 2.2 |
| OFAR1 61 | 2008 | I–II | FR-CLL, RS | 50 (20) | 50% | 20% | NR | 6 month OS: 59% |
| OFAR2 51 | 2013 | I–II | R/R-CLL, RS | 102 (35) | 39% | 6.50% | NR | 6.6 |
| RCHOP 62 | 2014 | II | R/R CLL, RS | 60 (15) | 67% | 7% | 10 | 21 |
| OCHOP 18 | 2016 | II | RS | 37 (37) | 46% | 27% | 6.2 | 11.4 |
| REPOCH 63 | 2018 | Retrospective | RS | 46 (46) | 39% | NR | 3.5 | 5.9 |
| Selinexor 64 | 2017 | II | NHL, RS | 79 (8) | 40% | 0% | NR | NR |
| Pembrolizumab 65 | 2017 | II | R/R CLL, RS | 25 (9) | 44% | 11% | NR | 10.7 |
| CD19 CAR-T 66 67Cells | 2017 | II | R/R CLL, NHL, RS | 24 (5) | 66% | 33% | NR | NR |
CAR-T: Chimeric antigen receptor T cells; CR: Complete remission; FACPGM: Fludarabine, cytarabine, cyclophosphamide, cisplatin, GM-CSF; HyperCVXD: Cyclophosphamide, vincristine, liposomal daunorubicin, dexamethasone; NHL: Non-Hodgkin lymphoma; OCHOP: Ofatumumab, cyclophosphamide, doxorubicin, prednisone; OFAR: Oxaliplatin, fludarabine, cytarabine, rituximab; ORR: Overall response rate; OS: Overall survival. PFS: Progression-free survival; PLL: Prolymphocytic leukemia; R/R: Relpased/refractory; RCHOP: Rituximab, cyclophosphamide, doxorubicin, prednisone; RS: Richter syndrome; NR: Not reported.