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Diffuse Anterior Scleritis and Secondary Glaucoma as a Manifestation of Adult T-Cell Prolymphocytic Leukemia

Kommaraju, Kaushik R. BS, MS; Moroi, Sayoko MD, PhD; Demirci, Hakan MD

doi: 10.1097/ICO.0000000000000180
Case Report

Purpose: The aim of this study was to report a case of adult T-cell prolymphocytic leukemia (T-PLL), which presented with diffuse anterior scleritis and increased intraocular pressure.

Methods: This is a case report.

Results: A 57-year-old man presented with bilateral conjunctival hyperemia and chemosis associated with pain. He also complained of having fatigue, general weakness, and disequilibrium. His visual acuity was 20/20 bilaterally. His intraocular pressures were 34 mm Hg oculus dexter (OD) and 35 mm Hg oculus sinister (OS). His anterior segments were noted for moderate conjunctival injection, deep anterior chambers with +2 anterior chamber cells and flare, no abnormalities of iris structures, and mild nuclear sclerosis. His optic discs showed vertical cup-to-disc ratios of 0.4 oculus dexter and 0.7 oculus sinister. On B-scan ultrasonography, mild fluid in the sub-Tenon space and a thickening of the uvea were observed in both eyes. The patient was diagnosed with bilateral diffuse anterior scleritis and secondary glaucoma. Systemic evaluation revealed adult T-PLL with pulmonary, abdominal, and pelvic adenopathy; pleural effusion; and central nervous system (CNS) involvement. He was treated with systemic alemtuzumab and intrathecal alternating cytarabine/hydrocortisone and methotrexate/hydrocortisone. His lymphadenopathy decreased in size and the pleural effusion, scleritis, and secondary glaucoma resolved; however, his CNS disease was unresponsive. He was therefore given craniospinal irradiation with a total dose of 29.4 Gy. Despite radiotherapy, the CNS disease progressed, and the patient died 19 months after the presentation.

Conclusions: Scleritis with secondary glaucoma, although usually associated with inflammatory conditions, can rarely be a manifestation of adult T-PLL.

*Medical School, William Carey University, Hattiesburg, MS; and

Department of Ophthalmology and Visual Sciences, W.K. Kellogg Eye Center, University of Michigan, Ann Arbor, MI.

Reprints: Hakan Demirci, MD, Department of Ophthalmology and Visual Sciences, University of Michigan, W.K. Kellogg Eye Center, 1000 Wall St, Ann Arbor, MI 48105 (e-mail:

The authors have no funding or conflicts of interest to disclose.

Supported by Mrs and Mr Witham.

Received January 29, 2014

Accepted May 06, 2014

Copyright © 2014 Wolters Kluwer Health, Inc. All rights reserved.