التفاصيل البيبلوغرافية
| العنوان: |
Erdheim-Chester disease with chorioretinal and orbital involvement: a case report. |
| Alternate Title: |
Doença de Erdheim-Chester com envolvimento coriorretiniano e orbitário: relato de caso. (Portuguese) |
| المؤلفون: |
Cabuk, Kubra Serefoglu, Tellioglu, Adem, Karabulut, Gamze Ozturk, Nacaroglu, Senay Asik, Fazil, Korhan, Elverdi, Tuğrul, Taskapili, Muhittin, Karslioglu, Safak |
| المصدر: |
Arquivos Brasileiros de Oftalmologia; 2024, Vol. 87 Issue 5, p1-4, 4p |
| مصطلحات موضوعية: |
ERDHEIM-Chester disease, ORBITAL diseases, VISION disorders, LYMPHATIC diseases, RETINAL diseases, HISTIOCYTOSIS, LEG pain |
| Abstract (English): |
A 42-year-old woman presented with bilateral proptosis, chemosis, leg pain, and vision loss. Orbital, chorioretinal, and multi-organ involvement of Erdheim- Chester disease, a rare non-Langerhans histiocytosis, with a negative BRAF mutation was diagnosed based on clinical, radiological, and pathological findings. Interferon-alpha-2a (IFNα-2a) was started, and her clinical condition improved. However, 4 months later, she had vision loss with a history of IFNα- 2a cessation. The same therapy was administered, and her clinical condition improved. The Erdheim-Chester disease is a rare chronic histiocytic proliferative disease that requires a multidisciplinary approach and can be fatal if left untreated because of multisystemic involvements. [ABSTRACT FROM AUTHOR] |
| Abstract (Portuguese): |
Uma mulher de 42 anos apresentou proptose bi lateral, quemose, dor nas pernas e perda de visão. Com base em achados clínicos, radiológicos e patológicos, foi diag nosticada doença de Erdheim-Chester com acometimento orbitário, coriorretiniano e multiorgânico. Trata-se de uma rara histiocitose não Langerhans negativa para a mutação BRAF. Foi iniciado tratamento com interferon alfa-2a (IFNα-2a) e o quadro clínico melhorou. No entanto, quatro meses depois, a paciente apresentou perda visual após a cessação do IFNα-2a. A mesma terapia foi administrada novamente e sua condição clínica melhorou novamente. A doença de Erdheim-Chester é uma doença proliferativa histiocítica crônica rara que necessita de uma abordagem multidisciplinar e pode ser fatal se não tratada, devido a envolvimentos multissistêmicos. [ABSTRACT FROM AUTHOR] |
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