Diffuse anterior scleritis and secondary glaucoma as a manifestation of adult T-cell prolymphocytic leukemia.

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.