Clinical characteristics of patients with pcCD30+LPDs who developed extracutaneous disease
| Characteristic . | C-ALCL (n = 30) . | LyP (n = 13) . | Overall (N = 43) . |
|---|---|---|---|
| Sex (male:female) | 21:9 | 10:3 | 31:12 |
| Age at diagnosis, y | |||
| Median | 56 | 46 | 53 |
| Range | 26-79 | 31-77 | 26-79 |
| Initial extent | |||
| Solitary (T1) | 16 (54) | 0 (0) | 16 (37) |
| Localized (T2) | 7 (23) | 2 (15) | 9 (21) |
| Generalized (T3) | 7 (23) | 11 (85) | 18 (42) |
| Initial therapy for cutaneous lesions | |||
| RT | 12 (40) | 0 (0) | 12 (28) |
| CHOP | 5 (17) | 0 (0) | 5 (12) |
| MTX | 2 (7) | 2 (15) | 4 (9) |
| UVB/PUVA | 1 (3) | 1 (8) | 2 (5) |
| Excision | 7 (23) | 0 (0) | 7 (16) |
| Expectative | 3 (10) | 10 (77) | 13 (30) |
| Initial response rates for cutaneous lesions | 25 (83) | 4 (31) | 29 (67) |
| CR | 2 (7) | 7 (54) | 9 (21) |
| PR | 3 (10) | 2 (15) | 5 (12) |
| NR | |||
| Median time to extracutaneous relapse (range), mo | 27 (5-264) | 60 (7-230) | 35 (5-264) |
| TNM classification: node | |||
| N1 | 11 (37) | 2 (15) | 13 (30) |
| N2 | 12 (40) | 8 (62) | 20 (47) |
| N3 | 7 (23) | 3 (23) | 10 (23) |
| TNM classification: metastases | 24 (80) | 8 (62) | 32 (74) |
| M0 | 6 (20) | 5 (38) | 11 (26) |
| M1 | |||
| Therapy | |||
| Anthracycline-based chemotherapy (+RT/SCT*) | 25 (83) | 9 (69) | 34 (79) |
| RT | 3 (10) | 1 (8) | 4 (9) |
| None† | 2 (7) | 3 (23) | 5 (12) |
| Results | |||
| CR | 19 (63) | 7 (54)‡ | 26 (61) |
| PR | 3 (10) | 1 (8) | 4 (9) |
| NR | 8 (27) | 5 (38) | 13 (30) |
| Current status | |||
| Alive without disease | 11 (37) | 6 (46) | 17 (40) |
| Alive with disease | 2 (6) | 2 (15) | 4 (9) |
| Died of lymphoma | 14 (47) | 5 (39) | 19 (44) |
| Died of other cause | 3 (10) | 0 (0) | 3 (7) |
| Median follow-up (range), mo | 63.5 (12-325) | 134 (14-360) | 81 (12-360) |
| 5-y PFS, %§ | 32 | 54 | 39 |
| 5-y Cumulative incidence of DSD, %§ | 51 | 38.5 | 47 |
| 5-y OS, %§ | 41 | 61.5 | 47 |
| Characteristic . | C-ALCL (n = 30) . | LyP (n = 13) . | Overall (N = 43) . |
|---|---|---|---|
| Sex (male:female) | 21:9 | 10:3 | 31:12 |
| Age at diagnosis, y | |||
| Median | 56 | 46 | 53 |
| Range | 26-79 | 31-77 | 26-79 |
| Initial extent | |||
| Solitary (T1) | 16 (54) | 0 (0) | 16 (37) |
| Localized (T2) | 7 (23) | 2 (15) | 9 (21) |
| Generalized (T3) | 7 (23) | 11 (85) | 18 (42) |
| Initial therapy for cutaneous lesions | |||
| RT | 12 (40) | 0 (0) | 12 (28) |
| CHOP | 5 (17) | 0 (0) | 5 (12) |
| MTX | 2 (7) | 2 (15) | 4 (9) |
| UVB/PUVA | 1 (3) | 1 (8) | 2 (5) |
| Excision | 7 (23) | 0 (0) | 7 (16) |
| Expectative | 3 (10) | 10 (77) | 13 (30) |
| Initial response rates for cutaneous lesions | 25 (83) | 4 (31) | 29 (67) |
| CR | 2 (7) | 7 (54) | 9 (21) |
| PR | 3 (10) | 2 (15) | 5 (12) |
| NR | |||
| Median time to extracutaneous relapse (range), mo | 27 (5-264) | 60 (7-230) | 35 (5-264) |
| TNM classification: node | |||
| N1 | 11 (37) | 2 (15) | 13 (30) |
| N2 | 12 (40) | 8 (62) | 20 (47) |
| N3 | 7 (23) | 3 (23) | 10 (23) |
| TNM classification: metastases | 24 (80) | 8 (62) | 32 (74) |
| M0 | 6 (20) | 5 (38) | 11 (26) |
| M1 | |||
| Therapy | |||
| Anthracycline-based chemotherapy (+RT/SCT*) | 25 (83) | 9 (69) | 34 (79) |
| RT | 3 (10) | 1 (8) | 4 (9) |
| None† | 2 (7) | 3 (23) | 5 (12) |
| Results | |||
| CR | 19 (63) | 7 (54)‡ | 26 (61) |
| PR | 3 (10) | 1 (8) | 4 (9) |
| NR | 8 (27) | 5 (38) | 13 (30) |
| Current status | |||
| Alive without disease | 11 (37) | 6 (46) | 17 (40) |
| Alive with disease | 2 (6) | 2 (15) | 4 (9) |
| Died of lymphoma | 14 (47) | 5 (39) | 19 (44) |
| Died of other cause | 3 (10) | 0 (0) | 3 (7) |
| Median follow-up (range), mo | 63.5 (12-325) | 134 (14-360) | 81 (12-360) |
| 5-y PFS, %§ | 32 | 54 | 39 |
| 5-y Cumulative incidence of DSD, %§ | 51 | 38.5 | 47 |
| 5-y OS, %§ | 41 | 61.5 | 47 |
PFS was defined as time between date of response to therapy for first extracutaneous lesion(s) and first event (progressive disease following the European Organization of Research and Treatment of Cancer, International Society for Cutaneous Lymphomas, and United States Cutaneous Lymphoma Consortium consensus guidelines for pcCD30+LPDs) or death of any cause. Time intervals of cumulative incidence of DSD were determined with time between date of development of extracutaneous disease and date of death from lymphoma or treatment toxicity, and OS was defined as time between date of development of extracutaneous disease to date of death from any cause. Data are presented as N (%) unless otherwise indicated.
CHOP, cyclophosphamide, doxorubicin, vincristine, and prednisone; M0, no evidence of extracutaneous non–lymph node disease; M1, extracutaneous non–lymph node disease present; MTX, methotrexate; N1, involvement of 1 peripheral lymph node region that drains an area of current or prior skin involvement; N2, involvement of 2 or more peripheral lymph node regions or involvement of any lymph node region that does not drain an area of current or prior skin involvement; N3, involvement of central lymph nodes; SCT, stem cell transplantation; UVB/PUVA, ultraviolet B/psoralen and ultraviolet A.
SCT included autologous SCT as well as allogeneic SCT.
Spontaneous regression, refused therapy, or death before start of treatment.
CR refers to complete remission of extracutaneous localizations.
After extracutaneous development.