How is risk determined for Langerhans cell histiocytosis (LCH)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Answer

Risk stratification for Langerhans cell histiocytosis (LCH) requires evaluation of the site and extent of disease involvement and response to therapy. Involvement of the risk organs, including bone marrow, spleen, liver, and possibly lungs, substantially increases the risk of mortality. Pediatric patients with high-risk LCH have a nearly 90% chance of survival, but the prognosis worsens if there is progression of the disease during the first 12 weeks of therapy. Some studies implicate somatic mutation of BRAF-V600E in high-risk features, disease progression, permanent injury, and poor short-term chemotherapy response. [59] This mutation may be associated with a two-fold increase in the risk of treatment failure.


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