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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

12/11/2013

Pubmed ID

24322503

DOI

10.1097/MPH.0000000000000085

Scopus ID

2-s2.0-84920602594   3 Citations

Abstract

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 ME

Authors

Kristin M. Bingen PhD Professor in the Pediatrics department at Medical College of Wisconsin
Patricia A. Donohoue MD Chief, Professor in the Pediatrics department at Medical College of Wisconsin
Jeffrey S. Karst PhD Assistant Professor in the Pediatrics department at Medical College of Wisconsin
Mohit Maheshwari MD Associate Professor in the Radiology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Child
Cytarabine
Histiocytosis, Langerhans-Cell
Humans
Immunoglobulins, Intravenous
Magnetic Resonance Imaging
Male
Neurodegenerative Diseases