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» Expert Histio Net » For Professionals » Guidelines » LCH childhood » Clinical Classification  · 

Clinical Classification

Author(s): Euro Histio Net Work Group for LCH Guidelines (see introduction page), Created: 2011/03/10, last update: 2014/02/09

Treatment recommendations are based on site and extension of the disease which is why it is important to distinguish between single system LCH and multisystem LCH.

Single System LCH (SS-LCH)

One organ/system involved (uni- or multifocal):

  • Bone: unifocal (single bone) or multifocal (> 1 bone)
  • Skin
  • Lymph node (not the draining lymph node of another LCH lesion)
  • Lungs
  • Hypothalamic-pituitary / Central nervous system
  • Other (e.g. thyroid, thymus)

Multisystem LCH (MS-LCH)

Two or more organs/systems involved:

  • With involvement of “Risk Organs” (Hematopoietic system, spleen, and/or liver)
  • Without involvement of “Risk Organs”

Note: Other systems have also been proposed in order to classify disease extension. In particular the so called “activity score” has been proposed [Donadieu et al 2004a] and is still in use in some countries such as in France. This system provides a score (0 no activity, up to 5 maximum activity) for each organ or system possibly affected by LCH. The score can be measured at diagnosis as at any follow-up visit. A total score > 6 is considered as unfavorable, as well as any worsening of previous scores.

See here the definition of organ involvement.
Possibly involved organs: bone, skin, hematopoietic system, spleen, liver, lung, mucosea, eye, ear, pituitary, CNS, other; risk organs; special sites

[Donadieu et al 2004a] Donadieu J, Piguet C, Bernard F, Barkaoui M, Ouache M, Bertrand Y, Ibrahim H, Emile JF, Hermine O, Tazi A, Genereau T, Thomas C: A new clinical score for disease activity in Langerhans cell histiocytosis. Pediatric blood & cancer 2004, 43: 770 [PMID: 15390280]