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Edgar JD, Smyth AE, Pritchard J, Lammas D, Jouanguy E, Hague R, Novelli V, Dempsey S, Sweeney L, Taggart AJ, O'hara D, Casanova JL, Kumararatne DS |
Interferon-gamma receptor deficiency mimicking Langerhans' cell histiocytosis. |
The Journal of pediatrics 2001, 139: 600 |
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Two patients who were initially given a diagnosis of Langerhans' cell histiocytosis on the basis of the clinical, radiologic, and biopsy findings had mycobacterial infection subsequently identified. The correct diagnosis of dominant partial interferon-gamma receptor deficiency was established. |
Egeler RM, Neglia JP, Puccetti DM, Brennan CA, Nesbit ME |
Association of Langerhans cell histiocytosis with malignant neoplasms. |
Cancer 1993 Feb 1; 71: 865 |
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The association of Langerhans cell histiocytosis (LCH) with a malignant neoplasm is rare and generally has been the subject of isolated case reports. |
Ehrhardt MJ, Karst J, Donohoue PA, Maheshwari M, McClain KL, Bingen K, Kelly ME |
Recognition and Treatment of Concurrent Active and Neurodegenerative Langerhans Cell Histiocytosis: A Case Report. |
Journal of pediatric hematology/oncology 2013 Dec 7; |
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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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