Characteristics and treatments of the 37 patients with ECD
| Patient no. . | Sex/age, y . | Disease duration, mo . | Topography of lesions . | Therapy . | Duration of IFN treatment, mo . |
|---|---|---|---|---|---|
| 1 | M/39 | 30.0 | B, MS, LV, H, S, O, CNS, P, L, R | None | ND |
| 2 | M/49 | 0.0 | B, MS, P, R | None | ND |
| 3 | F/73 | 0.0 | B | None | ND |
| 4 | F/59 | 19.1 | B, MS, LV, H, L, R | None | ND |
| 5 | M/47 | 47.7 | B, MS, H, S, O, CNS | None | ND |
| 6 | F/79 | 79.7 | B, LV | None | ND |
| 7 | M/70 | 0.0 | B, MS, LV, H, O, R | None | ND |
| 8 | M/49 | 0.5 | B, LV, H, R | None | ND |
| 9 | M/53 | 0.0 | B, S, P, R | None | ND |
| 10 | M/70 | 0.0 | MS, LV, H, CNS | None | ND |
| 11 | M/45 | 40.3 | B, MS, LV, CNS, L | None | ND |
| 12 | M/80 | 1.8 | B, H, R | None | ND |
| 13 | F/42 | 0.0 | B, LV, H, L | None | ND |
| 14 | M/69 | 0.5 | B, LV, H, S, O, CNS, R | None | ND |
| 15 | M/44 | 6.5 | B, S, L, R | None | ND |
| 16 | M/60 | 4.9 | B, MS, LV, H, S, CNS, L, R | IFN-6 MIU 3×/wk | 4.8 |
| 17 | M/71 | 335.4 | B, MS, LV, CNS, P, L | IFN-3 MIU 3×/wk | 48.8 |
| 18 | F/62 | 28.3 | B, MS, H, CNS, L | IFN-9 MIU 3×/wk | 28.3 |
| 19 | M/61 | 85.7 | B, R | Pegylated IFN 135 μg/wk | 84.7 |
| 20 | M/80 | 24.4 | B, MS, H, L | Pegylated IFN 180 μg/wk | 16.8 |
| 21 | M/56 | 9.5 | B, MS, LV, H, R | Prednisone 12 mg/d + IFN-3 MIU 3×/wk | 8.9 |
| 22 | M/31 | 61.4 | B, MS, CNS, P | IFN-9 MIU 3×/wk | 12.3 |
| 23 | M/63 | 121.5 | B, LV, H, S, CNS, L, R | Pegylated IFN 180 μg/wk | 51.9 |
| 24 | M/56 | 5.3 | B, MS, P, R | IFN-3 MIU 3×/wk | 1.9 |
| 25 | M/74 | 36.9 | B, LV, H, L, R | Pegylated IFN 135 μg/wk | 28.6 |
| 26 | M/66 | 56.7 | B, MS, LV, H, S, O, CNS, L | IFN-9 MIU 3×/wk | 32.9 |
| 27 | M/63 | 12.0 | B, MS, LV, CNS, R | IFN-6 MIU 3×/wk | 7.1 |
| 28 | F/70 | 7.9 | B, LV, H, O, R | IFN-3 MIU 3×/wk | 6.0 |
| 29 | M/63 | 88.6 | B, LV, H, CNS, P, L | IFN-9 MIU 3×/wk | 20.6 |
| 30 | M/67 | 21.2 | B, MS, L | Pegylated IFN 135 μg/wk | 13.8 |
| 31 | M/61 | 37.0 | B, MS, LV, CNS, L, R | Pegylated IFN 90 μg/wk | 33.9 |
| 32 | F/56 | 44.2 | B | Pegylated IFN 180 μg/wk | 24.3 |
| 33 | F/21 | 53.1 | MS, P, L, R | Pegylated IFN 135 μg/wk | 24.2 |
| 34 | M/55 | 18.5 | B, MS, LV, H, S, O, CNS, L, R | IFN-9 MIU 3×/wk | 17.0 |
| 35 | M/52 | 176.0 | B, MS, S, O, R | IFN-6 MIU 3×/wk | 175.6 |
| 36 | F/66 | 38.4 | B, LV, H | Pegylated IFN 180 μg/wk | 31.2 |
| 37 | F/76 | 30.6 | B, MS, LV, H, R | Pegylated IFN 180 μg/wk | 28.3 |
| Patient no. . | Sex/age, y . | Disease duration, mo . | Topography of lesions . | Therapy . | Duration of IFN treatment, mo . |
|---|---|---|---|---|---|
| 1 | M/39 | 30.0 | B, MS, LV, H, S, O, CNS, P, L, R | None | ND |
| 2 | M/49 | 0.0 | B, MS, P, R | None | ND |
| 3 | F/73 | 0.0 | B | None | ND |
| 4 | F/59 | 19.1 | B, MS, LV, H, L, R | None | ND |
| 5 | M/47 | 47.7 | B, MS, H, S, O, CNS | None | ND |
| 6 | F/79 | 79.7 | B, LV | None | ND |
| 7 | M/70 | 0.0 | B, MS, LV, H, O, R | None | ND |
| 8 | M/49 | 0.5 | B, LV, H, R | None | ND |
| 9 | M/53 | 0.0 | B, S, P, R | None | ND |
| 10 | M/70 | 0.0 | MS, LV, H, CNS | None | ND |
| 11 | M/45 | 40.3 | B, MS, LV, CNS, L | None | ND |
| 12 | M/80 | 1.8 | B, H, R | None | ND |
| 13 | F/42 | 0.0 | B, LV, H, L | None | ND |
| 14 | M/69 | 0.5 | B, LV, H, S, O, CNS, R | None | ND |
| 15 | M/44 | 6.5 | B, S, L, R | None | ND |
| 16 | M/60 | 4.9 | B, MS, LV, H, S, CNS, L, R | IFN-6 MIU 3×/wk | 4.8 |
| 17 | M/71 | 335.4 | B, MS, LV, CNS, P, L | IFN-3 MIU 3×/wk | 48.8 |
| 18 | F/62 | 28.3 | B, MS, H, CNS, L | IFN-9 MIU 3×/wk | 28.3 |
| 19 | M/61 | 85.7 | B, R | Pegylated IFN 135 μg/wk | 84.7 |
| 20 | M/80 | 24.4 | B, MS, H, L | Pegylated IFN 180 μg/wk | 16.8 |
| 21 | M/56 | 9.5 | B, MS, LV, H, R | Prednisone 12 mg/d + IFN-3 MIU 3×/wk | 8.9 |
| 22 | M/31 | 61.4 | B, MS, CNS, P | IFN-9 MIU 3×/wk | 12.3 |
| 23 | M/63 | 121.5 | B, LV, H, S, CNS, L, R | Pegylated IFN 180 μg/wk | 51.9 |
| 24 | M/56 | 5.3 | B, MS, P, R | IFN-3 MIU 3×/wk | 1.9 |
| 25 | M/74 | 36.9 | B, LV, H, L, R | Pegylated IFN 135 μg/wk | 28.6 |
| 26 | M/66 | 56.7 | B, MS, LV, H, S, O, CNS, L | IFN-9 MIU 3×/wk | 32.9 |
| 27 | M/63 | 12.0 | B, MS, LV, CNS, R | IFN-6 MIU 3×/wk | 7.1 |
| 28 | F/70 | 7.9 | B, LV, H, O, R | IFN-3 MIU 3×/wk | 6.0 |
| 29 | M/63 | 88.6 | B, LV, H, CNS, P, L | IFN-9 MIU 3×/wk | 20.6 |
| 30 | M/67 | 21.2 | B, MS, L | Pegylated IFN 135 μg/wk | 13.8 |
| 31 | M/61 | 37.0 | B, MS, LV, CNS, L, R | Pegylated IFN 90 μg/wk | 33.9 |
| 32 | F/56 | 44.2 | B | Pegylated IFN 180 μg/wk | 24.3 |
| 33 | F/21 | 53.1 | MS, P, L, R | Pegylated IFN 135 μg/wk | 24.2 |
| 34 | M/55 | 18.5 | B, MS, LV, H, S, O, CNS, L, R | IFN-9 MIU 3×/wk | 17.0 |
| 35 | M/52 | 176.0 | B, MS, S, O, R | IFN-6 MIU 3×/wk | 175.6 |
| 36 | F/66 | 38.4 | B, LV, H | Pegylated IFN 180 μg/wk | 31.2 |
| 37 | F/76 | 30.6 | B, MS, LV, H, R | Pegylated IFN 180 μg/wk | 28.3 |
Data for age is at serum sampling; disease duration is from histologic diagnosis of ECD. Therapy is at the time of serum sampling.
B indicates long bones; MS, maxillary sinus; LV, large vessels; H, heart; S, skin, O, orbit; CNS, central nervous system; P, pituitary gland; L, lungs; R, retroperitoneal; MIU, million international units; and ND, none determined.