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

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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]

References:

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Last Updated: February 25, 2014
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