Clinical and molecular characteristics of BL cohorts
| . | Sporadic BL . | Endemic BL . |
|---|---|---|
| Total cases, n | 78 | 89 |
| Median age at diagnosis (range), y | 8 (0-19) | 7 (2-16) |
| Sex, n (%) | ||
| Male | 61 (78.2) | 57 (64.0) |
| U | 5 (6.4) | 1 (1.1) |
| Tumor stage, n (%) | ||
| High (3 or 4) | 30 (38.5) | 58 (65.2) |
| U | 38 (48.7) | 8 (9.0) |
| CSF involvement, n (%) | ||
| Y | 1 (1.3) | 10 (11.2) |
| U | 28 (36.0) | 25 (28.1) |
| BM involvement, n (%) | ||
| Y | 18 (23.1) | 8 (9.0) |
| U | 17 (21.8) | 33 (37.1) |
| CNS involvement, n (%) | ||
| Y | 4 (5.1) | ND |
| U | 23 (29.5) | |
| EBV positive, n (%) | ||
| Y | 4 (5.1) | 76 (85.4) |
| U | 22 (28.2) | 13 (14.6) |
| MYC translocation, n (%) | ||
| Y | 73 (93.6) | 89 (100) |
| U | 1 (1.3) | 0 (0) |
| Treatment, n (%) | ||
| FAB/LMB 96 Protocol | 63 (81) | — |
| Group A | 1 | — |
| Group B | 44 | — |
| Group C | 14 | — |
| Group unknown | 4 | — |
| Malawi 28 d protocol* | — | 78 (88) |
| With doxorubicin | — | 60 |
| Reduced protocol | — | 18 |
| No treatment | 1 (1) | 5 (6) |
| U | 14 (18) | 6 (7) |
| Follow-up | ||
| Number of cases with follow-up data (%) | 71 (91.0) | 71 (79.8) |
| Disease progression | ||
| Relapse/refractory disease, n | 10 | 22 |
| Median time to relapse (range), mo | 5.5 (2.8-6.7) | 5.1 (1.6-10.9) |
| DFS | ||
| Progression or death from lymphoma, n | 12 | 30 |
| Median time to event (range), mo | 4.83 (0.9-6.7) | 3.92 (0.03-10.9) |
| OS | ||
| Dead, n | 12 | 31 |
| Median time to death (range), mo | 4.42 (0.1-11.1) | 4.73 (0.03-14.6) |
| . | Sporadic BL . | Endemic BL . |
|---|---|---|
| Total cases, n | 78 | 89 |
| Median age at diagnosis (range), y | 8 (0-19) | 7 (2-16) |
| Sex, n (%) | ||
| Male | 61 (78.2) | 57 (64.0) |
| U | 5 (6.4) | 1 (1.1) |
| Tumor stage, n (%) | ||
| High (3 or 4) | 30 (38.5) | 58 (65.2) |
| U | 38 (48.7) | 8 (9.0) |
| CSF involvement, n (%) | ||
| Y | 1 (1.3) | 10 (11.2) |
| U | 28 (36.0) | 25 (28.1) |
| BM involvement, n (%) | ||
| Y | 18 (23.1) | 8 (9.0) |
| U | 17 (21.8) | 33 (37.1) |
| CNS involvement, n (%) | ||
| Y | 4 (5.1) | ND |
| U | 23 (29.5) | |
| EBV positive, n (%) | ||
| Y | 4 (5.1) | 76 (85.4) |
| U | 22 (28.2) | 13 (14.6) |
| MYC translocation, n (%) | ||
| Y | 73 (93.6) | 89 (100) |
| U | 1 (1.3) | 0 (0) |
| Treatment, n (%) | ||
| FAB/LMB 96 Protocol | 63 (81) | — |
| Group A | 1 | — |
| Group B | 44 | — |
| Group C | 14 | — |
| Group unknown | 4 | — |
| Malawi 28 d protocol* | — | 78 (88) |
| With doxorubicin | — | 60 |
| Reduced protocol | — | 18 |
| No treatment | 1 (1) | 5 (6) |
| U | 14 (18) | 6 (7) |
| Follow-up | ||
| Number of cases with follow-up data (%) | 71 (91.0) | 71 (79.8) |
| Disease progression | ||
| Relapse/refractory disease, n | 10 | 22 |
| Median time to relapse (range), mo | 5.5 (2.8-6.7) | 5.1 (1.6-10.9) |
| DFS | ||
| Progression or death from lymphoma, n | 12 | 30 |
| Median time to event (range), mo | 4.83 (0.9-6.7) | 3.92 (0.03-10.9) |
| OS | ||
| Dead, n | 12 | 31 |
| Median time to death (range), mo | 4.42 (0.1-11.1) | 4.73 (0.03-14.6) |
BM, bone marrow; CNS, central nervous system; CSF, cerebrospinal fluid; EBV, Epstein-Barr virus; FAB-LMB, French-American-British/Lymphome Mains de Burkitt; U, unknown; Y, yes.
Endemic BL patients were treated with low-intensity 28-d regimen,29,30 receiving cyclophosphamide (cyclo), vincristine (vcr), prednisolone (pred), and doxorubicin (dox). Eighteen endemic BL patients received a reduced protocol (4 had cyclo only, 6 cyclo+vcr, and 8 cyclo+vcr+pred).