Recognition and treatment of concurrent active and neurodegenerative langerhans cell histiocytosis: a case report. J Pediatr Hematol Oncol 2015 Jan;37(1):e37-40
Date
12/11/2013Pubmed ID
24322503DOI
10.1097/MPH.0000000000000085Scopus ID
2-s2.0-84920602594 (requires institutional sign-in at Scopus site) 7 CitationsAbstract
Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation with subsequent tissue damage often requiring chemotherapy. Neurodegenerative LCH presents with neuromuscular, cognitive, and behavioral alterations typically occurring years after diagnosis of active LCH. We present a male child with a 4-year history of growth arrest, polyuria, polydipsia, recurrent otitis media, and seborrheic dermatitis. Cutaneous biopsies confirmed LCH and chemotherapy was initiated. During treatment for active LCH he developed neuropsychiatric decline. White matter changes on brain MRI were consistent with neurodegenerative LCH. Treatment was changed to cytarabine and intravenous immunoglobulin. After 1 year of therapy the patient experienced neuropsychological improvement.
Author List
Ehrhardt MJ, Karst J, Donohoue PA, Maheshwari M, McClain KL, Bingen K, Kelly MEAuthors
Kristin M. Bingen PhD Professor in the Pediatrics department at Medical College of WisconsinJeffrey S. Karst PhD Associate Professor in the Pediatrics department at Medical College of Wisconsin
Mohit Maheshwari MD Professor in the Radiology department at Medical College of Wisconsin
MESH terms used to index this publication - Major topics in bold
ChildCytarabine
Histiocytosis, Langerhans-Cell
Humans
Immunoglobulins, Intravenous
Magnetic Resonance Imaging
Male
Neurodegenerative Diseases
