Langerhans Cell Histiocytosis
Associated Genetic Biomarkers
NCI Definition: A neoplastic proliferation of Langerhans cells which contain Birbeck granules by ultrastructural examination. Three major overlapping syndromes are recognized: eosinophilic granuloma, Letterer-Siwe disease, and Hand-Schuller-Christian disease. The clinical course is generally related to the number of organs affected at presentation. (WHO, 2001) 
Langerhans cell histiocytosiss most frequently harbor alterations in BRAF .
BRAF Mutation, BRAF V600E, BRAF Exon 15 Mutation, and BRAF Codon 600 Missense are the most common alterations in langerhans cell histiocytosis .
There are 6 clinical trials for langerhans cell histiocytosis, of which 5 are open and 1 is completed or closed. Of the trials that contain langerhans cell histiocytosis as an inclusion criterion, 2 are phase 1 (1 open), 2 are phase 2 (2 open), 1 is phase 3 (1 open), and 1 is no phase specified (1 open).
BRAF is the most frequent gene inclusion criterion for langerhans cell histiocytosis clinical trials .
Donor lymphocytes, allogeneic hematopoietic stem cell transplantation, and cobimetinib are the most common interventions in langerhans cell histiocytosis clinical trials.
Significant Genes in Langerhans Cell Histiocytosis
BRAF is altered in 35.0% of langerhans cell histiocytosis patients .
BRAF is an inclusion eligibility criterion in 2 clinical trials for langerhans cell histiocytosis, of which 2 are open and 0 are closed. Of the trials that contain BRAF status and langerhans cell histiocytosis as inclusion criteria, 1 is phase 1 (1 open) and 1 is phase 2 (1 open) .
FLT3 is an inclusion eligibility criterion in 1 clinical trial for langerhans cell histiocytosis, of which 0 are open and 1 is closed. Of the trial that contains FLT3 status and langerhans cell histiocytosis as inclusion criteria, 1 is phase 1 (0 open) .
2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 6. This dataset does not represent the totality of the genetic landscape; see paper for more information.