Table 1 Clinical characteristics of adult LCH patients at the time of diagnosis
| No.(ratio %) | |
|---|---|
| Total patient | 36 |
| Gender | |
| Male | 21 (58.3%) |
| Female | 15 (41.7%) |
| Age at diagnosis (yrs) | |
| Median (range) | 35.5 (18–57) |
| Disease classification | |
| Multifocal single system (SS-m) | 5 (13.9%) |
| Multiple system without risk organ involvement (RO-) | 20 (55.6%) |
| Multiple system with risk organ involvement (RO+) | 11 (30.6%) |
| Liver involvement | 10 (27.8%) |
| Spleen involvement | 1 (2.8%) |
| Bone marrow (BM) involvement | 1 (2.8%) |
| Other organ/system involvement | |
| Pulmonary involvement |
21 (58.3%) 20/21 manifesting with pulmonary function disturbance. |
| Bone involvement | 31 (86.1%) |
| Bone aching | 18 (50%) |
| Lytic lesions | 31 (86.1%) |
| Lesion sites | |
| Skull bone | 22 (61.1%) |
| Vertebra | 18 (50%) |
| Pelvis | 13 (36.1%) |
| Ribs | 11 (30.6%) |
| Extremity bones | 8 (22.2%) |
| Central nervous system involvement | 25 (69.4%) |
| Pituitary | 23 (63.9%) |
| Other sitesa | 2 (5.6%) |
| Lymph nodes involvement | 21 (58.3%) |
| CNS-risk organ involvement | 8 (22.2%) |
| Thyroid involvement | 7 (19.4%) |
| BRAFV600E mutation | |
| No. of patients tested | 19 |
| No. of positivity | 4 (21.1%) |