Analytic validity refers to how well a genetic assessment measures the property or characteristic it is intended to measure. In the case of family history, analytic validity refers to the accuracy of the reported family history information. In the case of a test for a specific mutation, analytic validity refers to the accuracy of a genetic test in identifying the presence or absence of the mutation. The technical accuracy and reliability of the testing procedure and the quality of the laboratory...
Added text about how pulmonary involvement is present in approximately 25% of children with multisystem low-risk and high-risk LCH; however, a multivariate analysis of pulmonary disease in multisystem LCH did not show pulmonary disease to be an independent prognostic factor (cited Ronceray et al. as reference 22).
Revised text to state that the incidence of diabetes insipidus was lower in patients treated with more intensive chemotherapy regimens on the JLSG-96 and JLSG-02 studies in Japan (8.9% for multisystem patients) than on the LCH-I and LCH-II studies (14.2%) (cited 2001 Gadner et al. and 2008 Gadner et al. as references 32 and 33, respectively).
Treatment of Childhood LCH
Revised text to state that curettage only, curettage plus injection of methylprednisolone, or radiation therapy may be used to treat single skull lesions of the frontal, parietal, or occipital regions, or single lesions of any other bone (cited Gramatovici et al. as reference 9).
Added text to state that it is important to note that the cited studies included lungs as risk organs. However, subsequent analyses have shown that lung involvement lacks prognostic significance (cited Ronceray et al. as reference 24).
Revised text to state that treatment with vinblastine with or without corticosteroids for patients with central nervous system (CNS) mass lesions (20 patients; mainly pituitary) demonstrated an objective response in 15 patients, with 5 of 20 patients demonstrating a complete response and 10 of 20 patients demonstrating a partial response.
Treatment of Adult LCH
Added text about a retrospective review of 58 adult LCH patients that reported on the efficacy and toxicities of treatment with vinblastine/prednisone, cladribine, and cytarabine (cited Cantu et al. as reference 16).
Added text to state that a case report suggests some benefit to treating neurodegenerative CNS LCH disease with infliximab, a tumor necrosis factor-alpha inhibitor (cited Chohan et al. as reference 26).
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.
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February 25, 2014
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