Table 1 Clinical characteristics of adult LCH patients at the time of diagnosis
| Â | 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%) |