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Table 2 Comparison between the present and former classification of LCH [21]

From: Merkel cell polyomavirus and Langerhans cell neoplasm

Classification

Prevalence

Present

Former

LCH-RO (+)

MS

Letterer-Siwe disease

10%

LCH-RO (−)

MS

Hand–Schüller–Christian disease

20%

SS

Eosinophilic granuloma

70%

  1. LCH-RO (+): LCH involving at least one high-risk organ; LCH-RO (−) LCH involving a no high-risk organ; SS-LCH: LCH involving a single organ system; MS-LCH: LCH involving multiple organ systems