Table 1.

Clinical classification of LCH

Children
Clinical groupDescription
Multisystem Two or more systems involved 
 With risk organ involvement  Involvement of liver, spleen, or bone marrow 
 Without risk organ involvement  Without involvement of liver, spleen, or bone marrow 
Single system Only 1 system involved 
 Single site Skin, bone, lymph node, thyroid, thymus 
 Multiple sites Multifocal bone disease 
 Special site Skull-base lesion with intracranial extension or vertebral lesion with intraspinal soft tissue extension 
Pulmonary LCH Isolated lung disease 
CNS-LCH Tumorous lesions 
 Neurodegenerative disease
 LACI
 LACS 
Adults 
Unifocal Solitary lesion involving any organ 
Single-system pulmonary Isolated lung involvement, predominantly smoking related 
Single-system multifocal ≥1 lesion involving any organ 
Multisystem ≥2 organ/system involvement 
Children
Clinical groupDescription
Multisystem Two or more systems involved 
 With risk organ involvement  Involvement of liver, spleen, or bone marrow 
 Without risk organ involvement  Without involvement of liver, spleen, or bone marrow 
Single system Only 1 system involved 
 Single site Skin, bone, lymph node, thyroid, thymus 
 Multiple sites Multifocal bone disease 
 Special site Skull-base lesion with intracranial extension or vertebral lesion with intraspinal soft tissue extension 
Pulmonary LCH Isolated lung disease 
CNS-LCH Tumorous lesions 
 Neurodegenerative disease
 LACI
 LACS 
Adults 
Unifocal Solitary lesion involving any organ 
Single-system pulmonary Isolated lung involvement, predominantly smoking related 
Single-system multifocal ≥1 lesion involving any organ 
Multisystem ≥2 organ/system involvement 
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