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- Title
Response to mitogen‐activated protein kinase inhibition of neurodegeneration in Langerhans cell histiocytosis monitored by cerebrospinal fluid neurofilament light as a biomarker: a pilot study.
- Authors
Henter, Jan‐Inge; Kvedaraite, Egle; Martín Muñoz, Daniel; Cheng Munthe‐Kaas, Monica; Zeller, Bernward; Nystad, Tove A.; Björklund, Caroline; Donnér, Isabella; Lourda, Magda; Zetterberg, Henrik; Blennow, Kaj; Herold, Nikolas; Gavhed, Désirée; von Bahr Greenwood, Tatiana
- Abstract
Response to mitogen-activated protein kinase inhibition of neurodegeneration in Langerhans cell histiocytosis monitored by cerebrospinal fluid neurofilament light as a biomarker: a pilot study Keywords: Langerhans cell histiocytosis; central nervous system; neurodegeneration; mitogen-activated protein kinase inhibition; neurofilament light chain protein EN Langerhans cell histiocytosis central nervous system neurodegeneration mitogen-activated protein kinase inhibition neurofilament light chain protein 248 254 7 12/27/21 20220101 NES 220101 Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia with highly variable clinical presentation.1 Granulomatous lesions of bone, skin, and lungs (particularly in adults) are most common, but the liver, spleen, bone marrow, and central nervous system (CNS) may also be affected.1 CNS involvement (CNS LCH) often causes endocrinopathies, most commonly diabetes insipidus (DI), but may also cause a debilitating slowly-progressive neurodegeneration.1,2 Notably, a population-based study reported that at least 24% of all children with LCH develop signs of neurodegenerative CNS LCH (ND-CNS-LCH) on magnetic resonance imaging (MRI).3 Thus, a strategy for early detection, treatment, and monitoring of ND-CNS-LCH is imperative. Central nervous system, neurodegeneration, Langerhans cell histiocytosis, mitogen-activated protein kinase inhibition, neurofilament light chain protein GLO:1XW/01jan22:bjh17781-fig-0001.jpg PHOTO (COLOR): 1 Neurofilament (NFL) levels in the cerebrospinal fluid (CSF) in the five children with central nervous system (CNS) affection of Langerhans cell histiocytosis (LCH) before and after initiation of treatment with mitogen-activated protein kinase pathway inhibitors (MAPKi).
- Subjects
LANGERHANS-cell histiocytosis; MITOGEN-activated protein kinases; CEREBROSPINAL fluid; DIABETES insipidus; CYTOPLASMIC filaments; GLIAL fibrillary acidic protein
- Publication
British Journal of Haematology, 2022, Vol 196, Issue 1, p248
- ISSN
0007-1048
- Publication type
Article
- DOI
10.1111/bjh.17781