Clinical characteristics and follow-up data
| . | SPTL-AB . | SPTL-GD . | P . |
|---|---|---|---|
| Total patients | 63 (76) | 20 (24) | — |
| Median age, y (range) | 36 (9-79) | 59 (13-79) | .022 |
| Sex | ns | ||
| Male | 21 (33) | 7 (35) | — |
| Female | 42 (67) | 13 (65) | — |
| Ratio M/F | 0.5 | 0.54 | — |
| Presenting skin lesions | — | ||
| Nodules / plaques | 63 (100) | 20 (100) | ns |
| Ulceration | 4 (6) | 9 (45) | <.001 |
| Extent of cutaneous involvement | ns | ||
| Single lesion | 8 (13) | 2 (10) | — |
| Regional | 6 (9) | 3 (15) | — |
| Multifocal | 49 (78) | 15 (75) | — |
| Staging | — | ||
| B-symptoms | 37 (59) | 13 (65) | ns |
| Lab abnormalities | 29 (46) | 11 (55) | ns |
| Abnormal CT scan | 5 (8) | 2 (10) | ns |
| Abnormal bone marrow | 12 (19) | 7 (35) | ns |
| Hemophagocytic syndrome* | 11 (17) | 9 (45) | .004 |
| Treatment | ns | ||
| CHOP or CHOP-like therapy | 31 (49) | 14 (70) | — |
| Immunosuppressive therapy | 24 (38) | 2 (10) | — |
| Radiotherapy | 3 (5) | 1 (5) | — |
| Surgery | 2 (3) | 0 (0) | — |
| No therapy | 2 (3) | 3 (15) | — |
| Unknown | 1 (2) | — | |
| Result initial treatment | ns | ||
| Complete remission | 42 (67) | 6 (30) | — |
| Partial remission | 8 (13) | 7 (35) | — |
| No response | 6 (10) | 2 (10) | — |
| Progressive disease | 6 (10) | 5 (25) | — |
| Status at last follow-up | < .001 | ||
| Alive and well | 39 (62) | 4 (20) | — |
| Alive with disease | 15 (24) | 1 (5) | — |
| Died of lymphoma | 8 (13) | 15 (75) | — |
| Died of other cause | 1 (2) | 0 (0) | — |
| Survival | — | ||
| 5-y overall survival | (82) | (11) | < .001 |
| 5-y disease specific survival | (85) | (11) | < .001 |
| . | SPTL-AB . | SPTL-GD . | P . |
|---|---|---|---|
| Total patients | 63 (76) | 20 (24) | — |
| Median age, y (range) | 36 (9-79) | 59 (13-79) | .022 |
| Sex | ns | ||
| Male | 21 (33) | 7 (35) | — |
| Female | 42 (67) | 13 (65) | — |
| Ratio M/F | 0.5 | 0.54 | — |
| Presenting skin lesions | — | ||
| Nodules / plaques | 63 (100) | 20 (100) | ns |
| Ulceration | 4 (6) | 9 (45) | <.001 |
| Extent of cutaneous involvement | ns | ||
| Single lesion | 8 (13) | 2 (10) | — |
| Regional | 6 (9) | 3 (15) | — |
| Multifocal | 49 (78) | 15 (75) | — |
| Staging | — | ||
| B-symptoms | 37 (59) | 13 (65) | ns |
| Lab abnormalities | 29 (46) | 11 (55) | ns |
| Abnormal CT scan | 5 (8) | 2 (10) | ns |
| Abnormal bone marrow | 12 (19) | 7 (35) | ns |
| Hemophagocytic syndrome* | 11 (17) | 9 (45) | .004 |
| Treatment | ns | ||
| CHOP or CHOP-like therapy | 31 (49) | 14 (70) | — |
| Immunosuppressive therapy | 24 (38) | 2 (10) | — |
| Radiotherapy | 3 (5) | 1 (5) | — |
| Surgery | 2 (3) | 0 (0) | — |
| No therapy | 2 (3) | 3 (15) | — |
| Unknown | 1 (2) | — | |
| Result initial treatment | ns | ||
| Complete remission | 42 (67) | 6 (30) | — |
| Partial remission | 8 (13) | 7 (35) | — |
| No response | 6 (10) | 2 (10) | — |
| Progressive disease | 6 (10) | 5 (25) | — |
| Status at last follow-up | < .001 | ||
| Alive and well | 39 (62) | 4 (20) | — |
| Alive with disease | 15 (24) | 1 (5) | — |
| Died of lymphoma | 8 (13) | 15 (75) | — |
| Died of other cause | 1 (2) | 0 (0) | — |
| Survival | — | ||
| 5-y overall survival | (82) | (11) | < .001 |
| 5-y disease specific survival | (85) | (11) | < .001 |
Data are numbers (%) unless otherwise indicated.
— indicates not applicable; and ns, not significant.
Hemophagocytic syndrome was defined by the criteria of Henter et al.22