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Updated: May 5, 2026
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Recognition and treatment of concurrent active and neurodegenerative langerhans cell histiocytosis: a case report
Matthew J Ehrhardt1, Jeffrey Karst, Patricia A Donohoue
1*Midwest Center for Cancer and Blood Disorders †Medical College of Wisconsin and Children's Hospital of Wisconsin ‡Department of Psychology, Marquette University, Milwaukee, WI §Texas Children's Cancer and Hematology Centers, Baylor College of Medicine, Houston, TX.
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.
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