Screening for neurodegeneration in Langerhans cell histiocytosis with neurofilament light in plasma. (17th May 2022)

• Record Type: Journal Article
• Authors: Sveijer, Malin; von Bahr Greenwood, Tatiana; Jädersten, Martin; Kvedaraite, Egle; Zetterberg, Henrik; Blennow, Kaj; Lourda, Magda; Gavhed, Désirée; Henter, Jan‐Inge
• Is Part Of: British journal of haematology. Volume 198:Number 4(2022)
• Page Start: 721
• Page End: 728
• Publication Date: 2022-05-17
• Subjects: central nervous system (CNS) -- Langerhans cell histiocytosis -- neurodegeneration -- neurofilament light chain protein (NFL); Hematology -- Periodicals; Blood -- Diseases -- Periodicals; 616.15
• DOI: 10.1111/bjh.18247
• Languages: English
• ISSNs: 0007-1048

Abstract

Summary: Patients with Langerhans cell histiocytosis (LCH) may develop progressive neurodegeneration in the central nervous system (ND‐CNS‐LCH). Neurofilament light protein (NFL) in cerebrospinal fluid (CSF) is a promising biomarker to detect and monitor ND‐CNS‐LCH. We compared paired samples of NFL in plasma (p‐NFL) and CSF in 10 patients (19 samples). Nine samples had abnormal CSF‐NFL (defined as ≥380 ng/l) with corresponding p‐NFL ≥ 2 ng/l. Ten samples had CSF‐NFL < 380 ng/l; eight (80%) with p‐NFL < 2 ng/l ( p < 0.001; Fisher's exact test). Thus, our results suggest that p‐NFL may be used to screen for ND‐CNS‐LCH. Further studies are encouraged, including the role of p‐NFL for monitoring of ND‐CNS‐LCH.