Abstract
Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis typically affecting multiple organ systems. We report 2 patients who presented with homonymous hemianopia and were ultimately diagnosed with biopsy-confirmed ECD. We review the spectrum of ECD and its treatment as well as histopathological and immunohistochemical differentiation from other histiocytic disorders.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Adult
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Antigens, CD / metabolism
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Antigens, Differentiation, Myelomonocytic / metabolism
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Brain / metabolism
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Brain / pathology
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Erdheim-Chester Disease / complications*
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Extracellular Matrix Proteins / metabolism
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Female
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Hemianopsia / diagnosis
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Hemianopsia / etiology*
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Humans
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Hyaluronan Receptors / metabolism
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Magnetic Resonance Imaging
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Male
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Middle Aged
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Visual Fields / physiology
Substances
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Antigens, CD
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Antigens, Differentiation, Myelomonocytic
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CD68 antigen, human
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Extracellular Matrix Proteins
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Hyaluronan Receptors
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hyaluronan-mediated motility receptor