TABLE 2.
Organ involvement | Data |
---|---|
Disease classification, n (%) | |
SSs‐LCH | 10 (11.6) |
SSm‐LCH | 22 (25.6) |
PLCH | 8 (9.3) |
MS‐LCH | 46 (53.5) |
Median number of involved organs, n (range) | 2 (1–6) |
Organ involvement, n (%) | |
Bone | 52 (60.5) |
Lung | 24 (27.9) |
Pituitary gland | 22 (25.6) |
Lymph node | 21 (24.4) |
Skin | 20 (23.3) |
Liver | 14 (16.3) |
Central nervous system | 13 (15.1) |
Soft tissue | 8 (9.3) |
Other | 12 (14.0) |
Risk organ involvement, n (%) | 17 (19.8) |
Special site involvement, n (%) | 22 (25.6) |
Permanent consequences, n (%) | |
Diabetes insipidus | 19 (22.0) |
Anterior pituitary dysfunction | 11 (12.8) |
LCH‐associated neurodegeneration | 3 (3.5) |
Note: Risk organs: liver, spleen, or hematopoietic system; Special sites: vertebral lesions with intraspinal or craniofacial bone lesions with soft tissue extensions (orbit, mastoid, sphenoid, or temporal bones).
Abbreviations: MS‐LCH, multisystem LCH; PLCH, primary pulmonary LCH; SSm‐LCH, single system with multi‐site LCH; SSs‐LCH, single system with single‐site LCH.