Evaluation and treatment of Langerhans cell histiocytosis patients with central nervous system abnormalities: Current views and new vistas.
E Ann YehJay GreenbergOussama AblaGiulia LongoniEli DiamondMichelle HermistonBrandon TranCarlos Rodriguez GalindoCarl E AllenKenneth L McClainnull nullPublished in: Pediatric blood & cancer (2017)
Central nervous system (CNS) involvement in Langerhans cell histiocytosis (LCH) can include mass lesions of the hypothalamic pituitary axis, choroid plexus, cerebrum, and cerebellum or magnetic resonance imaging (MRI) signal abnormalities of the cerebellum, pons, and basal ganglia. The term neurodegenerative (ND) CNS-LCH has been given to the MRI signal abnormalities and neurologic dysfunction, although initially patients may have no clinical symptoms. Standardized evaluations to better understand the natural history and response to therapy are needed. We propose guidelines for clinical, radiologic, and physiologic tests as a framework for developing the best methods of evaluation, which can then be tested in prospective treatment protocols.
Keyphrases
- magnetic resonance imaging
- contrast enhanced
- end stage renal disease
- single cell
- computed tomography
- ejection fraction
- chronic kidney disease
- blood brain barrier
- diffusion weighted imaging
- combination therapy
- cerebrospinal fluid
- preterm infants
- peritoneal dialysis
- mesenchymal stem cells
- replacement therapy
- physical activity
- patient reported outcomes
- ultrasound guided