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      Anterior Chamber Non-Hodgkin Lymphoma of the Iris Masquerading as Uveitis-Glaucoma-Hyphema Syndrome

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          Abstract

          Purpose: To report a case of iris non-Hodgkin lymphoma initially thought to be uveitis-glaucoma-hyphema (UGH) syndrome. Methods: We reviewed the clinical, radiographic, and histopathologic findings in a patient with recurrent hyphemas and increased ocular pressure who eventually was found to have a rapidly growing iris mass. Results: An 89-year-old man with a history of cataract extraction and mantle cell lymphoma developed recurrent hyphema, which was subsequently revealed to be due to an iris mass. A biopsy revealed non-Hodgkin lymphoma that could not be formally subclassified but was suspicious for mantle cell lymphoma. The tumor showed a partial response to ibrutinib. Conclusion: Iris lymphoma can masquerade as a cause of recurrent hyphema after cataract extraction. Ophthalmologists should be aware of this presentation, especially in patients with a history of lymphoma.

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          Most cited references7

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          Iris involvement by lymphoma: a review of 13 cases.

          To describe the clinical and histopathological features of lymphomas involving the iris. Retrospective, descriptive study. Fourteen eyes of 13 patients. Review of medical records. Clinical and histopathological findings. Median patient age was 58 years (range, 25-76 years). Seven patients had known systemic lymphoma of which five were of large B-cell type. Symptoms included blurred vision (8/14), eye redness (3/14) and eye pain (2/14). Four eyes had secondary elevated intraocular pressure. Anterior segment findings included anterior chamber cells (14/14 eyes), keratic precipitates (11/14 eyes), congestion of conjunctival/episcleral blood vessels (9/14), hyphaema (7/14), conjunctival/anterior epibulbar lymphoma (6/14), tumour-induced pseudohypopyon (4/14) and corneal oedema (3/14). Of 12 eyes with adequate view of iris details, clinically detectable iris thickening or visible mass was noted in all (12/12). There was concomitant involvement of ciliary body (8/14), choroid (5/14) and orbit (2/14). All patients had biopsy of conjunctiva, episclera, iris or ciliary body, confirming the diagnosis of lymphoma. Histopathological or cytopathological evaluation of iris or ciliary body showed high-grade lymphoma in 9/11 eyes in which it was performed. Five patients did not have long-term follow up and, of the other eight patients, three died from complications of systemic lymphoma during follow up ranging from 1 to 44 months. Lymphomatous involvement of the iris should be considered in the differential diagnosis of corticosteroid-resistant uveitis in middle-aged and elderly patients. Iris lymphoma tends to be high grade and usually develops in patients with known aggressive systemic lymphoma. © 2012 The Authors. Clinical and Experimental Ophthalmology © 2012 Royal Australian and New Zealand College of Ophthalmologists.
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            Is Open Access

            Tumors of the eye and ocular adnexa at the Philippine Eye Research Institute: a 10-year review

            Objective We aimed to identify and describe the clinicopathologic characteristics of tumors of the eye and ocular adnexa at the Philippine Eye Research Institute. Methods The pathology reports of specimens submitted to the Ocular Pathology Section of the Philippine Eye Research Institute over a 10-year period (2003–2012) were reviewed, and collected data was subjected to descriptive statistical tabulation and analysis. Results A total of 1,551 histologically confirmed tumors were included. Of these, 254 were from the conjunctiva (155 benign, 99 malignant), 530 from the eyelids (360 benign, 170 malignant), 394 were intraocular tumors (21 benign, 373 malignant), and 373 were from the orbit (231 benign, 142 malignant). Retinoblastoma was the most common tumor of the eye and ocular adnexa in the series, accounting for 43.2% of all malignancies. Squamous cell carcinoma was the most frequent cancer of the conjunctiva, while basal cell carcinoma and sebaceous gland carcinoma were the most common eyelid malignancies. Orbital malignancies had a bimodal age distribution, with rhabdomyosarcoma being most frequent in children, while in adults, lymphoma was most common, followed by lacrimal gland carcinomas. Conclusion The majority of tumors of the conjunctiva, eyelids, and orbit were benign, while most intraocular tumors were malignant, with retinoblastoma being the most common.
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              • Abstract: not found
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              I. Epidemiology of adult non-Hodgkin lymphoma

              P Boffetta (2011)
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                Author and article information

                Journal
                OOP
                OOP
                10.1159/issn.2296-4657
                Ocular Oncology and Pathology
                S. Karger AG
                2296-4681
                2296-4657
                2016
                October 2016
                12 May 2016
                : 2
                : 4
                : 230-233
                Affiliations
                Departments of aOphthalmology and Visual Science and bPathology, Yale School of Medicine, and cOphthalmic Oncology, Smilow Cancer Hospital, New Haven, Conn., USA
                Author notes
                *Alexander T. Nguyen, MD, Department of Ophthalmology and Visual Science, Yale School of Medicine, 40 Temple Street, Suite 3B, New Haven, CT 06510 (USA), E-Mail alexander.nguyen@yale.edu
                Article
                445119 PMC5091236 Ocul Oncol Pathol 2016;2:230-233
                10.1159/000445119
                PMC5091236
                27843901
                467194cc-c933-43bd-ae09-fbd03d2fad82
                © 2016 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 14 January 2016
                : 18 February 2016
                Page count
                Figures: 4, References: 9, Pages: 4
                Categories
                Case Series and Brief Reports

                Vision sciences,Ophthalmology & Optometry,Pathology
                Uveitis,Mantle cell lymphoma,Hyphema,Iris,Non-Hodgkin lymphoma

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