Changes to the Summary (02/03/2014)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Revised text to state that in tested flow-sorted CD1a cells from fresh lesions, 10 of 16 samples had a pathogenic BRAF mutation. Also revised text to state that nine cases had the BRAF V600E mutation, and one additional case had a novel mutation, BRAF 600 DLAT, which demonstrated upregulation of ERK; these authors could not identify any clinical characteristics associated with the BRAF mutant genotype.
Revised text to state that a total of six patients with multiorgan LCH that was resistant to other agents, including 2-CdA, have been reported to respond to treatment with clofarabine (cited Abraham et al. as reference 15).
Added text to state that another study confirmed an approximate 50% survival at 10 years and improved hemodynamic changes associated with pulmonary arterial hypertension, but did not alter pulmonary function testing or incidence of pulmonary edema (cited Le Pavec et al. as reference 4).
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.