A single-centre study can improve our knowledge of a rare disease! As exemplified by studies in Langerhans' cell histiocytosis

Acta Paediatr. 2005 Aug;94(8):1010-1. doi: 10.1111/j.1651-2227.2005.tb02037.x.


The main contribution of this clinical study of Langerhans' cell histiocytosis (LCH) is that it provides information on the adult outcome of patients diagnosed during childhood.

Conclusion: The authors show that most patients, sometimes despite their sequelae, undertake higher education and live as independent adults. This study also offers evidence that some sequelae are due to radiotherapy, which should therefore be excluded from the treatment of LCH. The analysis of causes of death shows the important role of haematological dysfunction.

Publication types

  • Comment

MeSH terms

  • Academic Medical Centers
  • Adolescent
  • Adult
  • Age Distribution
  • Child
  • Female
  • France / epidemiology
  • Health Knowledge, Attitudes, Practice*
  • Histiocytosis, Langerhans-Cell / diagnosis
  • Histiocytosis, Langerhans-Cell / epidemiology*
  • Histiocytosis, Langerhans-Cell / radiotherapy*
  • Humans
  • Incidence
  • Male
  • Prognosis
  • Rare Diseases
  • Retrospective Studies
  • Risk Assessment
  • Severity of Illness Index
  • Sex Distribution
  • Survival Analysis