Amiloidosis primaria (AL) con presentación orbitaria.Caso clínico y revisión de la literatura
- Jacqueline Agustino Rodríguez
- E. de Armas Ramos
- E. Medina Mesa
- K. Armas Domínguez
- R. Méndez Medina
- P. Ruiz de la Fuente Rodríguez
- A. Abreu Reyes
- F. Martín Barrera
ISSN: 0211-2698
Datum der Publikation: 2017
Nummer: 28
Seiten: 131-136
Art: Artikel
Andere Publikationen in: Archivos de la Sociedad Canaria de Oftalmología
Zusammenfassung
Amyloidosis is a set of diseases characterized by the deposition of amyloid substance, which can be localized or affect several tissues. The orbital form is infrequent and may manifest as a superficial form, a deep form or a combination of both. Clinical Case: A 69-year-old male with upper left eyelid injury, which has progressively increased in 3 years. He has a history of prostate cancer, type A thymoma pT2N0M0, adrenal hyperplasia, critical patient polyneuropathy, and hyperuricemia. CT and MRI of the orbit showed a mass in the lacrimal gland with infiltration of the musculature, which does not enhance after contrast injection and does not produce bone erosion. An incisional biopsy was performed and was reported as orbital amyloidosis. Further extensive studies identified a monoclonal IgG kappa paraproteinemia. It was diagnosed of orbital localized amyloidosis in probable relationship with MGUS (Monoclonal gammopathy of undetermined significance). Due to the morbidity of the patient, a conservative attitude was undertaken with observation and imaging follow-ups. Conclusions: Symptoms of the different forms of amyloidosis are very non-specific and further studies must be carried out if the presence of the amyloid material in the tissues is believed likely. Treatment will depend on the particular type of amyloidosis. On systemic cases, clinical management will focus in decreasing amyloid protein production or in increasing elimination. In the localized cases, it is possible to consider observation, surgery or palliative treatments.