Table 2.

Clinical Classification of LCH

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, other (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 
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, other (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 
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