Table 2. Clinical Classification of LCHa

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 one system involved 
  Single siteSkin, 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

CNS = central nervous system; LACI = LCH-associated abnormal CNS imaging; LACS = LCH-associated abnormal CNS symptoms; LCH = Langerhans cell histiocytosis.

aReprinted from Blood, Volume 135, Issue 16, Carlos Rodriguez-Galindo, Carl E. Allen, Langerhans cell histiocytosis, Pages 1319–1331, Copyright 2020, with permission from Elsevier.[34]

From: Langerhans Cell Histiocytosis Treatment (PDQ®)

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PDQ Cancer Information Summaries [Internet].
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