Table 1.
Demographic features, presentation, management, clinical course, and outcome
| RH | Poly-E |
Poly-N | DLBCL | ALL | P for heterogeneity* | ||
|---|---|---|---|---|---|---|---|
| Poly-E with EBVMCU | EBVMCU | ||||||
| Demographics | |||||||
| N | 31 | 21 | 16 | 30 | 40 | 122 | |
| Age (range), y | 67 (45-90) | 79 (58-101) | 79 (64-101) | 73 (48-93) | 77 (59-90) | 75 (45-101) | .12† |
| Male/female, n | 16/15 | 9/12 | 6/10 | 13/17 | 29/11 | 67/55 | .046‡ |
| Involved site, n | 31 | 21 | 16 | 30 | 40 | 122 | |
| Extranodal, n (%) | 0 (0) | 21 (100) | 16 (100) | 0 (0) | 14 (35) | 35 (29) | |
| Nodal, n (%) | 31 (100) | 0 (0) | 0 (0) | 30 (100) | 24 (60) | 85 (69) | |
| Nodal and extranodal, n (%) | 0 (0) | 0 (0) | 0 (0) | 0 (0) | 2 (5) | 2 (2) | < .0001, any nodal involvement‡ |
| Extent of disease, n | 31 | 21 | 16 | 23 | 33 | 108 | |
| Localized, n (%) | 20 (65) | 16 (76) | 14 (87) | 10 (44) | 12 (37) | 58 (54) | .01, local vs not† |
| Tongue, n | 0 | 4 | 4 | 0 | 0 | 0 | |
| Tonsil, n | 0 | 4 | 4 | 0 | 0 | 0 | |
| Oral/palate/nasopharynx, n | 0 | 3 | 1 | 0 | 0 | 0 | |
| Skin, n | 0 | 5 | 5 | 0 | 0 | 0 | |
| Bulky localized, n (%) | 1 (3) | 1 (5) | 0 (0) | 0 (0) | 3 (9) | 5 (5) | |
| Two sites, n (%) | 1 (3) | 2 (9) | 2 (13) | 0 (0) | 3 (9) | 6 (5) | |
| Multiple sites, n (%) | 1 (3) | 1 (5) | 0 (0) | 4 (17) | 1 (3) | 7 (6) | |
| Generalized, n (%) | 8 (26) | 1 (5) | 0 (0) | 9 (39) | 14 (42) | 32 (30) | |
| B symptoms, n (%) | 10 (32) | 0 (0) | 0 (0) | 8 (35) | 12 (36) | 30 (28) | .02‡ |
| Management, n | 24 | 18 | 14 | 16 | 20 | 78 | |
| No treatment, n (%) | 19 (79) | 10 (55) | 9 (64) | 6 (38) | 0 (0) | 35 (45) | < .0001, treated vs not‡ |
| Chemotherapy, n (%) | 5 (21) | 2 (11) | 1 (7) | 9 (56) | 16 (80) | 32 (41) | |
| Radiotherapy, n (%) | 0 (0) | 4 (22) | 3 (21) | 0 (0) | 1 (5) | 5 (6) | |
| Chemotherapy + radiotherapy, n (%) | 0 (0) | 1 (6) | 1 (7) | 1 (6) | 2 (10) | 4 (5) | |
| Bone marrow transplantation, n (%) | 0 (0) | 1 (6) | 0 (0) | 0 (0) | 1 (5) | 2 (3) | |
| Course, n | 21 | 18 | 14 | 16 | 20 | 75 | |
| Spontaneous resolution, n (%) | 14 (67) | 6 (28) | 5 (36) | 3 (20) | 0 (0) | 23 (30) | |
| Relapsing/remitting, n (%) | 0 (0) | 3 (17) | 3 (21) | 0 (0) | 0 (0) | 3 (4) | |
| Complete remission [% of treated], n (%) | 4 (19) [100] | 6 (38) [75] | 5 (36) [100] | 6 (38) [60] | 11 (55) [55] | 27 (36) [63] | < .0001‡ |
| Partial remission [% of treated], n (%) | 0 (0) [0] | 1 (5) [13] | 0 (0) [0] | 0 (0) [0] | 4 (20) [20] | 5 (7) [12] | |
| Stable persistent disease, n (%) | 3 (14) | 1 (5) | 1 (7) | 1 (6) | 0 (0) | 5 (7) | |
| Progressive disease, n (%) | 0 (0) | 1 (5) | 0 (0) | 6 (38) | 5 (25) | 12 (16) | |
| Histologic transformation, n (%) | 3 (9) | 0 (0) | 0 (0) | 3 (10) | 0 (0) | 6 (5) | .56‡ |
| to Poly-N, n | 1 | 0 | 0 | 0 | 0 | 1 | |
| to DLBCL, n | 0 | 0 | 0 | 3 | 0 | 3 | |
| to cHL, n | 2 | 0 | 0 | 0 | 0 | 2 | |
| Outcomes, n | 17 | 15 | 14 | 13 | 18 | 63 | |
| Died of disease, n (%) | 0 (0) | 1 (7) | 0 (0) | 4 (31) | 7 (39) | 12 (19 | |
| Died of complications of Tx, n (%) | 0 (0) | 0 (0) | 0 (0) | 1 (8) | 2 (11) | 3 (5) | |
| Died of unrelated cause, n (%) | 1 (6) | 6 (40) | 6 (43) | 3 (23) | 1 (6) | 11 (17) | |
| Median (range) follow-up, mo | 36 (2-132) | 38 (2-72) | 38 (2-60) | 71 (40-101) | 24 (1-68) | 41 (1-132) | .2§ |
| Median survival, mo | NR | 24 | 21 | 24 | 25 | 53 | .009§ |
ALL indicates acute lymphoblastic leukemia; Tx, treatment; and NR, (median) not reached.
*
Comparing RH vs Poly-E vs Poly-N vs DLBCL.
†
Kruskal-Wallis test.
‡
χ2 test.
§
Unadjusted log-rank test.