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Bilateral Proptosis as Initial Manifestation of Mantle Cell Lymphoma

      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.
      Figure thumbnail gr1
      FigureMarked bilateral proptosis with conjunctival edema.
      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.

      Discussion

      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.
      • Raffield M.
      • Sander C.A.
      • Yano T.
      • Jaffe E.S.
      Mantle cell lymphoma: an update.
      Mantle cell lymphoma is characterized by a t(11:14) chromosomal translocation with overproduction of cyclin D1.
      • Coupland S.E.
      • Hummel M.
      • Stein H.
      Ocular adnexal lymphomas: five case presentations and a review of the literature.
      Mantle cell lymphoma represents approximately 6% of all non-Hodgkins lymphomas. It has a male preponderance, affecting men twice as often as women, and usually affects middle-aged to elderly patients.
      • Sander B.
      Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers.
      • Looi A.
      • Gascoyne R.D.
      • Chhanabhai M.
      • Connors J.M.
      • Rootman J.
      • White V.A.
      Mantle cell lymphoma in the ocular adnexal region.
      Mantle cell lymphoma commonly presents with systemic symptoms of fever, night sweats, or unexplained weight loss.
      • Coupland S.E.
      • Hummel M.
      • Stein H.
      Ocular adnexal lymphomas: five case presentations and a review of the literature.
      Biopsy is often required for diagnosis, but other methods, including cytogenetic analysis with fluorescence in situ hybridization and polymerase chain reaction, can be employed.
      • Coupland S.E.
      • Hummel M.
      • Stein H.
      Ocular adnexal lymphomas: five case presentations and a review of the literature.
      Mantle cell lymphoma is typically widespread at time of diagnosis. It typically affects lymph nodes, spleen, Waldeyer's ring, and bone marrow,
      • Sander B.
      Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers.
      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.
      • Looi A.
      • Gascoyne R.D.
      • Chhanabhai M.
      • Connors J.M.
      • Rootman J.
      • White V.A.
      Mantle cell lymphoma in the ocular adnexal region.
      • Abdullah A.
      • Elsamaloty H.
      • Patel Y.
      • Chang J.
      CT and MRI findings with histopathologic correlation of a unique bilateral orbital mantle cell lymphoma in Graves' disease: a case report and brief review of the literature.
      Mantle cell lymphoma has an aggressive clinical course, with a mean survival of 3-5 years
      • Sander B.
      Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers.
      and an overall 5-year survival rate of 50%.
      • Coupland S.E.
      • Hummel M.
      • Stein H.
      Ocular adnexal lymphomas: five case presentations and a review of the literature.
      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.
      • Looi A.
      • Gascoyne R.D.
      • Chhanabhai M.
      • Connors J.M.
      • Rootman J.
      • White V.A.
      Mantle cell lymphoma in the ocular adnexal region.

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        Ocular adnexal lymphomas: five case presentations and a review of the literature.
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        Mantle cell lymphoma in the ocular adnexal region.
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        CT and MRI findings with histopathologic correlation of a unique bilateral orbital mantle cell lymphoma in Graves' disease: a case report and brief review of the literature.
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