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Langerhans Cell Histiocytosis Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Presentation of LCH in Children


A study of CNS-related permanent consequences (neuropsychologic deficits) in 14 of 25 LCH patients followed for a median of 10 years has been published.[41] Seven of these patients had diabetes insipidus and five patients had radiographic evidence of LCH CNS neurodegenerative changes.[41] Patients with craniofacial lesions had lower performance and verbal intelligence quotient scores than those with other LCH lesions.

Histological evaluation of these neurodegenerative lesions shows a prominent T-cell infiltration, usually in the absence of the CD1a-positive dendritic cells along with microglial activation and gliosis. The neurodegenerative form of the disease has been compared to a paraneoplastic inflammatory response.[37]


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  28. Marchand I, Barkaoui MA, Garel C, et al.: Central diabetes insipidus as the inaugural manifestation of Langerhans cell histiocytosis: natural history and medical evaluation of 26 children and adolescents. J Clin Endocrinol Metab 96 (9): E1352-60, 2011.
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  38. Fahrner B, Prosch H, Minkov M, et al.: Long-term outcome of hypothalamic pituitary tumors in Langerhans cell histiocytosis. Pediatr Blood Cancer 58 (4): 606-10, 2012.
  39. Prayer D, Grois N, Prosch H, et al.: MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis. AJNR Am J Neuroradiol 25 (5): 880-91, 2004.
  40. Wnorowski M, Prosch H, Prayer D, et al.: Pattern and course of neurodegeneration in Langerhans cell histiocytosis. J Pediatr 153 (1): 127-32, 2008.
  41. Mittheisz E, Seidl R, Prayer D, et al.: Central nervous system-related permanent consequences in patients with Langerhans cell histiocytosis. Pediatr Blood Cancer 48 (1): 50-6, 2007.

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Last Updated: February 25, 2014
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