Biomarkers in the cerebrospinal fluid and neurodegeneration in Langerhans cell histiocytosis

Abstract

CSF levels of NF-L, TAU, and GFAp appear to be elevated in CNS-LCH. It would be valuable if these markers were validated in order to serve as markers for early CNS-LCH, to monitor disease progression and to evaluate various treatment attempts for CNS-LCH.

CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH‐associated neurodegeneration and mass lesions

“…In contrast to a previous report, CSF concentrations of GFAP, tau, and phospho‐tau were not significantly increased in LCH‐ND in this study compared with the ALL control or ND control groups . We analyzed the concentration of GFAP and tau (total and phospho‐tau) in a subset of LCH and ALL cases from this series.…”

“…In contrast to a previous report, CSF concentrations of GFAP, tau, and phospho-tau were not significantly increased in LCH-ND in this study compared with the ALL control or ND control groups. 24 We analyzed the concentration of GFAP and tau (total and phospho-tau) in a subset of LCH and ALL cases from this series. We found the concentrations of GFAP, total tau, and phospho-tau in CSF were not significantly different between all cases of LCH with CNS involvement (n 5 40 cases of LCH-ND, LCH-CNS mass, or both), control ND cases (n 5 4), and control ALL cases (n 5 22) (total tau, P 5 .81; phospho tau, P 5 .19; GFAP, P 5 .21).…”

Prognostic value of brain injury biomarkers in acute encephalitis/encephalopathy

“…Biochemical markers of brain injury, including S‐100B, neuron‐specific enolase, glial fibrillary acidic protein (GFAP), neurofilaments, and tau protein, might be attractive tools for predicting clinical outcomes in AEE . S‐100B, a calcium‐binding protein produced predominantly by astrocytes in the brain, is implicated in the development and maintenance of the nervous system .…”

“…S‐100B, a calcium‐binding protein produced predominantly by astrocytes in the brain, is implicated in the development and maintenance of the nervous system . GFAP, which is expressed almost exclusively in astrocytes, constitutes a major part of their cytoskeleton . Tau protein is a microtubule‐associated protein that is particularly abundant in neuronal axons .…”

Adsorptive depletion of blood monocytes reduces the levels of circulating interleukin-17A in Langerhans cell histiocytosis

“…1 Prolonged disease activity is a risk factor for ND-LCH. 1 Potential biomarkers in cerebrospinal fluid (CSF) indicative of ND-LCH have been suggested (eg, neurofilament protein light chain [NF-L]), 2 and increased interleukin-17A (IL-17A) levels in blood and extracranial lesions of LCH patients have been associated with local and systemic inflammation. 3,4 Independently, IL-17A has been shown to disrupt the blood-brain barrier and increase production of reactive oxygen species in brain endothelial cells, pointing toward a crucial step for the development of experimental autoimmune encephalitis 5 and possibly also for ND-LCH.…”