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Table 1 Clinical characteristics of adult LCH patients at the time of diagnosis

From: The combination of methotrexate and cytosine arabinoside in newly diagnosed adult Langerhans cell histiocytosis: a prospective phase II interventional clinical trial

  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%)
  1. aother sites include hypothalamus in 1 cases and parenchyma near cranial basis in 1 case