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A 65-year-old African American woman presented to the ophthalmology clinic reporting a 2-month history of painless bilateral proptosis with acute vision loss in her right eye. She denied any prior ophthalmic history. Her known medical history was remarkable for hypertension, for which she did not take medications. On examination, her visual acuity was light perception only in her right eye and 20/40 in her left eye. No afferent pupillary defect was noted. Visual field by confrontation was imperceptible in her right eye and full in her left eye. Extraocular motility was markedly reduced in both eyes and intraocular pressures were elevated, measuring 29 mm Hg in the right eye and 30 mm Hg in the left eye.
External examination revealed marked bilateral proptosis with conjunctival edema (Figure) measuring 35 mm in the right eye and 30 mm in the left by Hertel exophthalmometry. Tense, bilateral upper eyelid edema, bilateral chemosis, and bilateral punctate epithelial erosions were present. No optic disc edema or pallor was noted. Funduscopic examination was within normal limits.
The patient also had prominent temporal wasting and marked lymphadenopathy of the cervical, submandibular, and submental lymph nodes. Upon further questioning, the patient endorsed a 20-pound weight loss over the previous 2 months. Given this constellation of findings and presentation, concern for systemic malignancy was high and she was subsequently admitted to the General Medicine service for a complete systemic work-up.
Laboratory evaluation revealed hematologic abnormalities of an anemia of 10.0 g/dL and a leukocytosis of 47.1 k/μL with 21% neutrophils and 63% lymphocytes. Basic metabolic panel was within normal limits. Head computed tomography was performed, revealing bilateral enlargement of lacrimal glands and proptosis without discrete masses. A lymph node biopsy was performed and revealed mantle cell lymphoma. In collaboration with the oncology service, the patient was offered chemotherapy with rituximab and cyclophosphamide. The patent refused treatment and left the hospital against medical advice.
The patient missed her follow-up examination 1 month after discharge. Upon contacting her family, it was reported that she died 2 weeks after leaving the hospital.
Mantle cell lymphoma gained acceptance as a distinct subtype of non-Hodgkins lymphoma in 1992 as a group of lymphomas that derive from the follicular mantle zone.
and is rare in the periocular region. Orbital lymphomas, defined as those that involve the lacrimal gland, orbital soft tissue, eyelids, and conjunctiva, are not common entities. In cases of orbital involvement, bilateral involvement is rare, occuring in 10%-17% of cases, and mantle cell origin is especially rare, occurring in 2%-7% of cases.
Orbital involvement denotes advanced mantle cell lymphoma, in which no definitive treatment exists. None of the authors have seen such an advanced presentation of bilateral proptosis as initial presentation of mantle cell lymphoma. Palliative options include chemotherapeutic regimens and possible allogenic bone marrow transplantation.
Authorship: All authors warrant they have participated sufficiently in the preparation of this manuscript. All authors accept responsibility for the content of this manuscript and have agreed on the decision to publish. This article is original and is not under consideration for publication by another journal and has not been published previously or presented in whole or in abstract form.