How is Langerhans cell histiocytosis (LCH) treated?

Updated: Jun 12, 2020
  • Author: Christopher R Shea, MD; Chief Editor: William D James, MD  more...
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Answer

No consensus exists for the optimal therapy for Langerhans cell histiocytosis (LCH), particularly in the case of multisystem organ disease. However, the Histiocyte Society has conducted a number of prospective, randomized control trials to study the effect of various chemotherapeutic regimens in the treatment of LCH, which have resulted in recommendations by the Histiocyte Society.

Generally, the choice of therapeutic regimen is based on disease severity. The International LCH Study of the Histiocyte Society proposes the stratification of LCH cases by the number of systems involved. They further categorize those cases with single-system involvement by the number of sites within that system (eg, monostotic vs polyostotic bone disease, solitary vs multiple lymph node involvement). In addition, the presence or the absence of risk-organ dysfunction is used to stratify patients with multisystemic disease; the presence of risk-organ dysfunction portends a poorer prognosis.

Notably, most trials for the treatment of LCH have been performed in pediatric populations, and their results have typically been extrapolated to adults. This has resulted in a call for more prospective, randomized trials looking specifically at the treatment of LCH in adults and whether changes in chemotherapeutic regimens and their duration may be appropriate. [62]


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