Table 1.
Main clinical characteristics of the ECD population (n = 32)
| n (%) | |
|---|---|
| BRAF V600E mutation | 20 (62.5) |
| Age at first symptoms (mean[SD]) | 49.6 [14.2] |
| Current treatment | |
| Steroid | 6 (18.8) |
| Interferon alpha | 2 (6.3) |
| Vemurafenib | 12 (37.5) |
| Cobimetinib | 4 (12.5) |
| Others | 5 (15.6) |
| Past treatment | |
| Steroid | 13 (40.6) |
| Interferon alpha | 24 (75) |
| Vemurafenib | 12 (37.5) |
| Cobimetinib | 7 (21.9) |
| Others | 13 (40.6) |
| Cerebral MRI patterns* | |
| MRI considered as normal | 8 (25) |
| Tumoral patterns | 19 (59.4) |
| Pseudo-degenerative patterns | 15 (46.9) |
| Vascular patterns | 7 (21.9) |
ECD Erdheim-Chester disease
*MRI patterns was described on the basis of prior publications (see reference Cohen-Aubart et al. [7])