Clinical characteristics of the GZL spectrum and polymorphic-EBV-DLBCL cohorts (N = 112)
| Variable . | GZL spectrum, n = 85 . | Polymorphic-EBV-L, n = 27 . | ||
|---|---|---|---|---|
| Group 0, n = 11 . | Bona fide (Group 1-2), n = 56 . | Group 3, n = 18 . | ||
| Center, n (%) | ||||
| Centre for Lymphoid Cancer | 1 (10) | 7 (13) | 15 (83) | 3 (11) |
| LYSA | 10 (90) | 49 (87) | 3 (17) | 24 (89) |
| Age | ||||
| Median (range), y | 26 (14-55) | 48 (19-90) | 66 (19-79) | 53 (16-79) |
| >60 y, n (%) | 0 (0) | 16 (33) | 12 (18) | 11 (41) |
| Sex, n (%) | ||||
| Female | 6 (55) | 30 (54) | 9 (50) | 8 (47) |
| Male | 5 (45) | 26 (46) | 9 (50) | 19 (53) |
| Mediastinal involvement, n (%) | n = 10 | n = 52 | n = 18 | n = 26 |
| Thymic | 8 (80) | 33 (63)* | 3 (17) | 7 (27) |
| Mediastinal, non-thymic | 0 (0) | 6 (12) | 4 (22) | 8 (31) |
| Nonmediastinal | 2 (20) | 13 (25) | 11 (61) | 11 (42) |
| Not available, n | 1 | 4 | 0 | 1 |
| Pathology group, n | Not applicable† | |||
| 0 | 11 | — | — | |
| 1 | — | 31 | — | |
| 2 | — | 25 | — | |
| 3 | — | — | 18 | |
| Hemoglobin | n = 11 | n = 48 | n = 17 | n = 21 |
| <12 g/dL, n (%) | 4 (36) | 24 (50) | 4 (24) | 12 (57) |
| Bulky disease (10 cm), n/N (%) | 2/11 (18) | 16/47 (34) | 4/18 (22) | 2/16 (12) |
| Ann Arbor stage, n (%) | n = 11 | n = 51 | n = 18 | n = 23 |
| 1-2 | 5 (45) | 24 (47) | 9 (50) | 8 (35) |
| 3-4 | 6 (55) | 27 (53) | 9 (50) | 15 (65) |
| Cases with extranodal involvement, n/N (%) | 6/11 (54) | 23/51 (45) | 9/18 (50) | 12/23 (52) |
| Spleen enlargement, n (%) | 2 (33) | 6 (26) | 0 (0) | 11 (91) |
| Liver, n (%) | 0 (0) | 4 (17) | 0 (0) | 4 (33) |
| Lung, n (%) | 3 (50) | 10 (44) | 1 (11) | 2 (16) |
| Bone marrow, n (%) | 1 (17) | 8 (35) | 3 (33) | 2 (16) |
| LDH > UNL, n/N (%) | 3/11 (27) | 26/45 (58) | 8/18 (44) | 10/21 (48) |
| aaIPI, n (%) | n = 11 | n = 47 | n = 18 | n = 22 |
| 0-1 | 8 (73) | 28 (60) | 10 (55) | 10 (45) |
| 2-3 | 3 (27) | 19 (40) | 8 (45) | 12 (55) |
| Variable . | GZL spectrum, n = 85 . | Polymorphic-EBV-L, n = 27 . | ||
|---|---|---|---|---|
| Group 0, n = 11 . | Bona fide (Group 1-2), n = 56 . | Group 3, n = 18 . | ||
| Center, n (%) | ||||
| Centre for Lymphoid Cancer | 1 (10) | 7 (13) | 15 (83) | 3 (11) |
| LYSA | 10 (90) | 49 (87) | 3 (17) | 24 (89) |
| Age | ||||
| Median (range), y | 26 (14-55) | 48 (19-90) | 66 (19-79) | 53 (16-79) |
| >60 y, n (%) | 0 (0) | 16 (33) | 12 (18) | 11 (41) |
| Sex, n (%) | ||||
| Female | 6 (55) | 30 (54) | 9 (50) | 8 (47) |
| Male | 5 (45) | 26 (46) | 9 (50) | 19 (53) |
| Mediastinal involvement, n (%) | n = 10 | n = 52 | n = 18 | n = 26 |
| Thymic | 8 (80) | 33 (63)* | 3 (17) | 7 (27) |
| Mediastinal, non-thymic | 0 (0) | 6 (12) | 4 (22) | 8 (31) |
| Nonmediastinal | 2 (20) | 13 (25) | 11 (61) | 11 (42) |
| Not available, n | 1 | 4 | 0 | 1 |
| Pathology group, n | Not applicable† | |||
| 0 | 11 | — | — | |
| 1 | — | 31 | — | |
| 2 | — | 25 | — | |
| 3 | — | — | 18 | |
| Hemoglobin | n = 11 | n = 48 | n = 17 | n = 21 |
| <12 g/dL, n (%) | 4 (36) | 24 (50) | 4 (24) | 12 (57) |
| Bulky disease (10 cm), n/N (%) | 2/11 (18) | 16/47 (34) | 4/18 (22) | 2/16 (12) |
| Ann Arbor stage, n (%) | n = 11 | n = 51 | n = 18 | n = 23 |
| 1-2 | 5 (45) | 24 (47) | 9 (50) | 8 (35) |
| 3-4 | 6 (55) | 27 (53) | 9 (50) | 15 (65) |
| Cases with extranodal involvement, n/N (%) | 6/11 (54) | 23/51 (45) | 9/18 (50) | 12/23 (52) |
| Spleen enlargement, n (%) | 2 (33) | 6 (26) | 0 (0) | 11 (91) |
| Liver, n (%) | 0 (0) | 4 (17) | 0 (0) | 4 (33) |
| Lung, n (%) | 3 (50) | 10 (44) | 1 (11) | 2 (16) |
| Bone marrow, n (%) | 1 (17) | 8 (35) | 3 (33) | 2 (16) |
| LDH > UNL, n/N (%) | 3/11 (27) | 26/45 (58) | 8/18 (44) | 10/21 (48) |
| aaIPI, n (%) | n = 11 | n = 47 | n = 18 | n = 22 |
| 0-1 | 8 (73) | 28 (60) | 10 (55) | 10 (45) |
| 2-3 | 3 (27) | 19 (40) | 8 (45) | 12 (55) |
Characteristics of the 56 bona fide GZL and 27 polymorphic-EBV-DLBCL (polymorphic-EBV-L) cases are presented. To provide a full characterization of the spectrum, the cohort also included 11 group 0 cases (cHL-like morphology with intense and diffuse CD20 expression on all tumor cells) and 18 group 3 cases (large B-cell morphology with intense and diffuse CD30 expression on all tumor cells).
aaIPI, age-adjusted International Prognostic Index; LDH, lactate dehydrogenase; UNL, upper limit of normal.
Two cases with a lung biopsy not included in DEA.
Groups for polymorphic-EBV-L are not applicable here because these cases are not defined as GZL in the WHO classification.1