Table 1.
Patient characteristics and outcomes of clinical trials and case series of PEL
| Reference | ||||||||
|---|---|---|---|---|---|---|---|---|
| 93 | 71 | 68 | 65 | 62 | 72 | 74 | 73 | |
| No. of patients | 301 | 51 | 28 | 11 | 8 | 20 | 7 | 6 |
| Year of study | 2001-2012 | 1996-2013 | 1993-2003 | 1987-2002 | 1987-2001 | 2000-2013 | 2010-2017 | 2020 |
| PEL, % | 100 | 67 | 100 | 100 | 0 | 18 | 100 | 67 |
| Solid PEL, % | 24 | 33 | 0 | 0 | 100 | 1 | 0 | 33 |
| Median age, y | 55 | 45 | 44 | 41 | 40 | 44 | NA | 38 |
| HIV, % | 67 | 100 | 100 | 100 | 100 | 100 | 100 | 100 |
| ART, % | 100 | 78 | NA | 20 | 100 | 100 | 100 | |
| Median (range) CD4+ T-cell count, cells per μL | NA | 204 (90-370) | 133 (5-756) | NA* | 125 (53-389) | NA | 231 (3-403) | |
| EBV, % | 50 | 66 | 71 | NA | NA | 73 | 60 | |
| KS, % | 28 | 49 | 67 | 27 | 25 | 75 | NA | 66 |
| MCD, % | 4 | 35 | 32 | NA | 30 | NA | 17 | |
| Receiving chemotherapy, % | 86 | 88† | 79 | 73 | 75 | 95‡ | 100 | 100 |
| Chemotherapy backbone | Various | CHOP-like | CHOP-like | CHOP | CHOP | DA-EPOCH | DA-EPOCH | DA-EPOCHR |
| CR, % | NA | 62 classic, 41 solid | 41 | 42 | NA | 53 | 71 | 50 |
| Median OS, mo | 6 | 10.2 | 6.2 | 6 | 11 | 22 | NA | Data not mature |
| OS rate, % | NA | 43 (classic) and 39 (EC) at 5y |
39 at 1y | NA | 40 at 5y | 47 cancer-specific survival at 3y | NA | 67 at 2y |
| EFS rate, % | NA | 71 (classic) and 100% (EC) DFS at 2y | NA | NA | NA | NA | 71 3y EFS | NA |
ART, antiretroviral therapy; CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; CR, complete remission; da-EPOCH, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin; DA-EPOCHR, DA-EPOCH and rituximab; DFS, disease-free survival; EFS, event-free survival; NA, data not available; OS, overall survival; y, years.
*
Only 37% had data.
†
12 received low-dose/no treatment.
‡
19 of 20 patients; 1 patient died before therapy.