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A 64-year-old woman presented with a 6-month history of progressive bilateral proptosis and conjunctival erythema. Her medical history was significant for hypothyroidism, treated with levothyroxine, as well as 2 forms of hematologic malignancy: retroperitoneal extranodal marginal zone lymphoma and indolent-phase T-cell prolymphocytic leukemia. She had received no treatment for her hematologic malignancies, as she had been asymptomatic since her diagnoses 6 years prior.
Ophthalmological examination revealed 27 mm of bilateral proptosis, conjunctival injection, chemosis, and bilateral lower eyelid retraction (Figure 1A).1 Visual acuity, color vision, and pupillary responses were normal. Extraocular motility was full in all positions of gaze, and there was no associated pain. Upper eyelid retraction was minimal. Von Graefe sign (delayed descent of the upper eyelid with initiation of downgaze) and lagophthalmos were absent. Results of slitlamp examination and dilated fundus examination were normal, including the appearance of both optic nerves.
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T-cell prolymphocytic leukemia infiltration of the extraocular muscles
C. Perform extraocular muscle biopsy
Thyroid-associated orbitopathy (TAO) is the most prevalent orbital disease.2 Patients typically present in their fourth or sixth decade of life and women are affected 5 times more than men.2 TAO is associated with autoimmune thyroid dysfunction: 90% of individuals with TAO have Graves hyperthyroidism, 1% have primary hypothyroidism, 3% have Hashimoto thyroiditis, and 5% are euthyroid.2 The most common signs are upper eyelid retraction (38% to 90%) and proptosis (65%), followed by von Graefe sign, lower eyelid retraction, and lagophthalmos.3,4 Active TAO may manifest with orbital pain and eyelid, conjunctival, or caruncular inflammation.4
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Corresponding Author: Evan Kalin-Hajdu, MD, Department of Ophthalmology, Université de Montréal, CUO Maisonneuve-Rosemont, 5415 Blvd de l’Assomption, Montréal, QC H1T 2M4, Canada (email@example.com).
Published Online: January 14, 2021. doi:10.1001/jamaophthalmol.2020.4679
Conflict of Interest Statement: None reported.
Additional Contributions: We acknowledge Jérémie Berdugo, MD, Department of Pathology and Cellular Biology, Université de Montréal, Montreal, Quebec, Canada, and Marie-Christine Carette, MD, Department of Hemato-oncology, Hôpital de Saint-Eustache, Saint-Eustache, Quebec, Canada, for their contributions to patient care and the writing of this article. They were not compensated. We thank the patient for granting permission to publish this information.
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