Demographic, clinical characteristics, and outcome of ECD patients
| . | All, n = 120 . | CH, n = 51 . | No CH, n = 69 . | P* . |
|---|---|---|---|---|
| Sex, M/F | 82/38 | 38/13 | 44/25 | .24 |
| Age at first symptoms (mean, SD), y | 53.12 (15.51) | 59.39 (13.12) | 48.48 (15.60) | <.0001† |
| Age at diagnosis (mean, SD), y | 57.12 (13.88) | 63.25 (10.80) | 52.51 (14.21) | <.0001† |
| Age at BM aspirate (mean, SD), y | 61.58 (13.00) | 67.00 (9.83) | 58.00 (13.71) | <.0001† |
| BRAF V600E, (%) | 70/111 (63) | 36/49 (73) | 34/62 (55) | .049† |
| Mixed histiocytosis, (%) | 20 (17) | 8 (16) | 12 (17) | 1.00 |
| Long bone involvement, (%) | 104 (87) | 42 (82) | 62 (90) | .28 |
| Cardiac involvement, (%) | 59 (49) | 27 (53) | 32 (46) | .46 |
| Vascular involvement, (%) | 67 (56) | 34 (67) | 33 (48) | .04† |
| Xanthelasma, (%) | 24 (20) | 10 (20) | 14 (20) | 1.00 |
| Diabetes insipidus, (%) | 34 (28) | 11 (22) | 23 (33) | .22 |
| CNS involvement, (%) | 42 (35) | 15 (29) | 27 (39) | .33 |
| Retro-orbital involvement, (%) | 24 (20) | 13 (25) | 11 (16) | .25 |
| Retroperitoneal involvement | 75 (63) | 38 (75) | 37 (54) | .02† |
| Myeloid malignancies, (%) | 18 (15) | 16 (31) | 2 (3) | <.0001 |
| Deaths, (%) | 8 (7) | 4 (8) | 4 (6) | .72 |
| Follow-up since diagnosis, mean (range), mo | 59 (1-236) | 52 (1-196) | 59 (1-273) | .39 |
| Follow-up since BM aspirate, mean (SD), mo | 13.44 (4.46) | 13.96 (4.83) | 13.06 (4.16) | .21 |
| IFN-α or PEG-IFN-α, (%) | 74 (62) | 31 (61) | 43 (62) | 1.00 |
| Targeted therapy, (%) | ||||
| BRAF inhibitor | 50 (42) | 28 (55) | 22 (33) | .02† |
| MEK inhibitor | 21 (18) | 9 (18) | 12 (17) | 1.00 |
| . | All, n = 120 . | CH, n = 51 . | No CH, n = 69 . | P* . |
|---|---|---|---|---|
| Sex, M/F | 82/38 | 38/13 | 44/25 | .24 |
| Age at first symptoms (mean, SD), y | 53.12 (15.51) | 59.39 (13.12) | 48.48 (15.60) | <.0001† |
| Age at diagnosis (mean, SD), y | 57.12 (13.88) | 63.25 (10.80) | 52.51 (14.21) | <.0001† |
| Age at BM aspirate (mean, SD), y | 61.58 (13.00) | 67.00 (9.83) | 58.00 (13.71) | <.0001† |
| BRAF V600E, (%) | 70/111 (63) | 36/49 (73) | 34/62 (55) | .049† |
| Mixed histiocytosis, (%) | 20 (17) | 8 (16) | 12 (17) | 1.00 |
| Long bone involvement, (%) | 104 (87) | 42 (82) | 62 (90) | .28 |
| Cardiac involvement, (%) | 59 (49) | 27 (53) | 32 (46) | .46 |
| Vascular involvement, (%) | 67 (56) | 34 (67) | 33 (48) | .04† |
| Xanthelasma, (%) | 24 (20) | 10 (20) | 14 (20) | 1.00 |
| Diabetes insipidus, (%) | 34 (28) | 11 (22) | 23 (33) | .22 |
| CNS involvement, (%) | 42 (35) | 15 (29) | 27 (39) | .33 |
| Retro-orbital involvement, (%) | 24 (20) | 13 (25) | 11 (16) | .25 |
| Retroperitoneal involvement | 75 (63) | 38 (75) | 37 (54) | .02† |
| Myeloid malignancies, (%) | 18 (15) | 16 (31) | 2 (3) | <.0001 |
| Deaths, (%) | 8 (7) | 4 (8) | 4 (6) | .72 |
| Follow-up since diagnosis, mean (range), mo | 59 (1-236) | 52 (1-196) | 59 (1-273) | .39 |
| Follow-up since BM aspirate, mean (SD), mo | 13.44 (4.46) | 13.96 (4.83) | 13.06 (4.16) | .21 |
| IFN-α or PEG-IFN-α, (%) | 74 (62) | 31 (61) | 43 (62) | 1.00 |
| Targeted therapy, (%) | ||||
| BRAF inhibitor | 50 (42) | 28 (55) | 22 (33) | .02† |
| MEK inhibitor | 21 (18) | 9 (18) | 12 (17) | 1.00 |