Skip to main content

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