Main clinical findings and follow-up data
| . | LyP . | Primary cutaneous CD30+ LTCL . | CD30+ LTCL in skin and regional lymph nodes . | Secondary cutaneous CD30+ LTCL . |
|---|---|---|---|---|
| Number | 118 | 79 | 11 | 11 |
| Sex (M:F) | 69:49 | 59:20 | 4:7 | 6:5 |
| Age (y) | ||||
| Median | 45.5 | 60 | 64 | 55 |
| Range | 4-88 | 16-89 | 27-74 | 7-87 |
| Extent of skin lesions | ||||
| Solitary | 0 | 42 (53%) | 7 (64%) | 1 (9%) |
| Regional | 15 (13%) | 20 (25%) | 3 (27%) | 1 (9%) |
| Generalized | 103 (87%) | 17 (22%) | 1 (9%) | 8 (82%) |
| Type of skin lesions | ||||
| Papule | 80 (68%) | 6 (7%) | 1 (9%) | 3 (27%) |
| Nodule | 4 (3%) | 23 (29%) | 0 | 5 (46%) |
| Papules and nodules | 26 (22%) | 0 | 0 | 0 |
| Tumor | 5 (4%) | 47 (60%) | 10 (91%) | 3 (27%) |
| Plaques | 3 (3%) | 3 (4%) | 0 | 0 |
| Spontaneous remission | ||||
| Absent | 0 | 46 (58%) | 8 (73%) | 6 (55%) |
| Partial | 0 | 17 (22%) | 1 (9%) | 5 (45%) |
| Complete | 118 (100%) | 16 (20%) | 2 (18%) | 0 |
| B symptoms at diagnosis | ||||
| Absent | 118 (100%) | 79 (100%) | 11 (100%) | 5 (45%) |
| Present | 0 | 0 | 0 | 6 (55%) |
| Initial therapy | ||||
| Radiotherapy | 5 (4%) | 38 (48%) | 0 | 2 (18%) |
| Multiagent chemotherapy | 0 | 6 (8%) | 9 (82%) | 9 (82%) |
| Excision | 0 | 15 (19%) | 1 (9%) | 0 |
| PUVA/UV-B | 41 (35%) | 3 (4%) | 0 | 0 |
| Other | 6 (5%) | 2 (2%) | 0 | 0 |
| None/topical steroids | 66 (56%) | 15 (19%) | 1 (9%) | 0 |
| Result of initial therapy | ||||
| Complete remission | 16 (14%) | 78 (99%) | 10 (91%) | 3 (27%) |
| Partial remission | 31 (26%) | 1 (1%) | 0 | 5 (55%) |
| No response | 61 (60%) | 0 | 1 (9%) | 3 (27%) |
| Disease-free survival (mo) | ||||
| Median | NR | 23 | 38 | 0 |
| Range | NR | 1-192 | 0-84 | 0-115 |
| Relapse | ||||
| Skin only | 111 (94%) | 32 (41%) | 4 (36%) | 2 (18%) |
| Regional lymph node | 5 (4%) | 5 (6%) | 1 (9%) | 1 (9%) |
| Systemic | 2 (2%) | 3 (4%) | 1 (9%) | 55% |
| Follow-up (mo) | ||||
| Median | 77 | 61 | 58 | 22 |
| Range | 12-350 | 13-288 | 15-284 | 6-133 |
| Current status | ||||
| No evidence of disease | 38 (32%) | 51 (65%) | 6 (55%) | 2 (18%) |
| Alive with disease | 73 (62%) | 13 (16%) | 3 (27%) | 1 (9%) |
| Died of lymphoma | 2 (2%) | 4 (5%) | 1 (9%) | 8 (73%) |
| Died of other cause | 5 (4%) | 11 (14%) | 1 (9%) | 0 |
| Disease-related survival | ||||
| 5 years | 100% | 96% | 91% | 24%* (44%) |
| 10 years | 100% | 96% | 91% | 24%* (24%) |
| Overall survival | ||||
| 5 years | 98% | 83% | 76% | 24%* (44%) |
| 10 years | 95% | 78% | 76% | 24%* (24%) |
| Calculated risk for extracutaneous disease | ||||
| 5 years | 2% | 9% | 20%† | 80%† |
| 10 years | 4% | 16% | 20%† | 80%† |
| . | LyP . | Primary cutaneous CD30+ LTCL . | CD30+ LTCL in skin and regional lymph nodes . | Secondary cutaneous CD30+ LTCL . |
|---|---|---|---|---|
| Number | 118 | 79 | 11 | 11 |
| Sex (M:F) | 69:49 | 59:20 | 4:7 | 6:5 |
| Age (y) | ||||
| Median | 45.5 | 60 | 64 | 55 |
| Range | 4-88 | 16-89 | 27-74 | 7-87 |
| Extent of skin lesions | ||||
| Solitary | 0 | 42 (53%) | 7 (64%) | 1 (9%) |
| Regional | 15 (13%) | 20 (25%) | 3 (27%) | 1 (9%) |
| Generalized | 103 (87%) | 17 (22%) | 1 (9%) | 8 (82%) |
| Type of skin lesions | ||||
| Papule | 80 (68%) | 6 (7%) | 1 (9%) | 3 (27%) |
| Nodule | 4 (3%) | 23 (29%) | 0 | 5 (46%) |
| Papules and nodules | 26 (22%) | 0 | 0 | 0 |
| Tumor | 5 (4%) | 47 (60%) | 10 (91%) | 3 (27%) |
| Plaques | 3 (3%) | 3 (4%) | 0 | 0 |
| Spontaneous remission | ||||
| Absent | 0 | 46 (58%) | 8 (73%) | 6 (55%) |
| Partial | 0 | 17 (22%) | 1 (9%) | 5 (45%) |
| Complete | 118 (100%) | 16 (20%) | 2 (18%) | 0 |
| B symptoms at diagnosis | ||||
| Absent | 118 (100%) | 79 (100%) | 11 (100%) | 5 (45%) |
| Present | 0 | 0 | 0 | 6 (55%) |
| Initial therapy | ||||
| Radiotherapy | 5 (4%) | 38 (48%) | 0 | 2 (18%) |
| Multiagent chemotherapy | 0 | 6 (8%) | 9 (82%) | 9 (82%) |
| Excision | 0 | 15 (19%) | 1 (9%) | 0 |
| PUVA/UV-B | 41 (35%) | 3 (4%) | 0 | 0 |
| Other | 6 (5%) | 2 (2%) | 0 | 0 |
| None/topical steroids | 66 (56%) | 15 (19%) | 1 (9%) | 0 |
| Result of initial therapy | ||||
| Complete remission | 16 (14%) | 78 (99%) | 10 (91%) | 3 (27%) |
| Partial remission | 31 (26%) | 1 (1%) | 0 | 5 (55%) |
| No response | 61 (60%) | 0 | 1 (9%) | 3 (27%) |
| Disease-free survival (mo) | ||||
| Median | NR | 23 | 38 | 0 |
| Range | NR | 1-192 | 0-84 | 0-115 |
| Relapse | ||||
| Skin only | 111 (94%) | 32 (41%) | 4 (36%) | 2 (18%) |
| Regional lymph node | 5 (4%) | 5 (6%) | 1 (9%) | 1 (9%) |
| Systemic | 2 (2%) | 3 (4%) | 1 (9%) | 55% |
| Follow-up (mo) | ||||
| Median | 77 | 61 | 58 | 22 |
| Range | 12-350 | 13-288 | 15-284 | 6-133 |
| Current status | ||||
| No evidence of disease | 38 (32%) | 51 (65%) | 6 (55%) | 2 (18%) |
| Alive with disease | 73 (62%) | 13 (16%) | 3 (27%) | 1 (9%) |
| Died of lymphoma | 2 (2%) | 4 (5%) | 1 (9%) | 8 (73%) |
| Died of other cause | 5 (4%) | 11 (14%) | 1 (9%) | 0 |
| Disease-related survival | ||||
| 5 years | 100% | 96% | 91% | 24%* (44%) |
| 10 years | 100% | 96% | 91% | 24%* (24%) |
| Overall survival | ||||
| 5 years | 98% | 83% | 76% | 24%* (44%) |
| 10 years | 95% | 78% | 76% | 24%* (24%) |
| Calculated risk for extracutaneous disease | ||||
| 5 years | 2% | 9% | 20%† | 80%† |
| 10 years | 4% | 16% | 20%† | 80%† |