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.
This is a case report.
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.
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: firstname.lastname@example.org).
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