Interferon as a treatment for Erdheim-Chester Disease: a case report and literature review
Keywords:
Erdheim-Chester disease, ECD, Pegylated interferon-alfaAbstract
Erdheim-Chester disease (ECD) is a rare systemic non-Langerhans histiocytosis. Generally, the affected organs include bone, the pituitary gland, orbits, lungs, heart, skin, liver, spleen, retroperitoneum and kidneys. The radiological findings may be helpful for diagnosis guidance. Tissue biopsy should be obtained for pathologic diagnosis. This case report describes the case of a 41-year-old Thai man who presented bilateral progressive proptosis and blindness in the right eye. Incisional biopsy from the right eye was pathologically reviewed and combined with radiologic signs, leading to a diagnosis of ECD. The patient was treated with high-dose pegylated interferon-alfa. After six months of the treatment course, his clinical status improved, but imaging manifestations were slightly changed. This report also includes a literature review of ECD in the aspect of pathogenesis, clinical manifestations, diagnostic evaluation and management.
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