70 Jpn J Ophthalmol Vol 53: 61–76, 2009

Received: May 28, 2008 / Accepted: August 21, 2008Correspondence to: Kenya Yuki, Department of Ophthalmology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japane-mail: glaucoma.keio@gmail.com

DOI 10.1007/s10384-008-0605-z

References

1. Sotozono C, Ang LP, Koizumi N, et al. New grading system for the evaluation of chronic ocular manifestations in patients with Stevens-Johnson syndrome. Ophthalmology 2007;114:1294–1302.

2. Tsai JH, Derby E, Holland EJ, Khatana AK. Incidence and preva-lence of glaucoma in severe ocular surface disease. Cornea 2006;25:530–532.

3. Ytteborg J, Dohlman CH. Corneal edema and intraocular pressure. II. Clinical results. Arch Ophthalmol 1965;74:477–484.

4. Shaikh NM, Shaikh S, Singh K, Manche E. Progression to end-stage glaucoma after laser in situ keratomileusis. J Cataract Refract Surg 2002;28:356–359.

5. Rootman DS, Insler MS, Thompson HW, et al. Accuracy and precision of the Tono-Pen in measuring intraocular pressure after keratoplasty and epikeratophakia and in scarred corneas. Arch Ophthalmol 1988;106:1697–1700.

Adult T-Cell Leukemia Presenting with Episcleritis and

Secondary Glaucoma

Adult T-cell leukemia (ATL) results from infection by the human T-cell lymphotrophic virus type 1 (HTLV-1), and the major ocular manifestations are opportunistic infection and infi ltrative lesions by leukemic cells.1,2 Episcleritis is a rare ocular manifestation of ATL.3 We treated an ATL patient who presented with episcleritis and the complica-tion of secondary glaucoma.

Case Report

A 64-year-old woman had suffered for 1 month from head-aches and conjunctivitis in both eyes that were resistant to the 0.02% fl uorometholone and levofl oxacin administered at her neighborhood eye hospital (Fig. 1a, b). Elevation of intraocular pressure (IOP) in both eyes was found on reex-

Figure 1a–e. Photographs of the anterior segment of the right (a) and left (b) eyes before chemo-therapy, and the right (c) and left (d) eyes after chemotherapy. Seven days after the initiation of chemotherapy, dilation of con-junctival and episcleral vessels completely disappeared. e Abnor-mal multilobed lymphocytes are present in the peripheral blood smear (arrow). Bar = 10 μm.

LETTERS 71

amination. Three days later, she was referred to our hospi-tal. At our initial examination, her visual acuity was 1.0 OU. IOP was 38 mmHg OD and 43 mmHg OS. The conjunctival and episcleral vessels were moderately dilated (Fig. 1a, b). There was no proptosis, and the eye movements were full. The anterior chamber of both eyes showed normal depth and no infl ammatory cells. Gonioscopy showed no abnor-malities in either eye. The vitreous, retina, and optic disc of both eyes were normal. The patient had no signifi cant medical history. Goldmann perimetry showed no scotoma and no constriction of the visual fi elds. From these fi ndings, we suspected that she was a steroid responder with episcle-ritis and carotid cavernous fi stula.

After admission to our hospital, the topical 0.02% fl uo-rometholone was discontinued and topical antiglaucoma drugs, 0.5% timolol, 0.005% latanoprost, and oral acetazol-amide, were begun. Computed tomography, magnetic resonance imaging, and cerebral angiography of the head showed no abnormalities. However, laboratory tests showed an elevation of the white blood cell count and liver enzymes. Abnormal multilobed lymphocytes were detected in periph-eral blood smears (Fig. 1e). In addition, the peripheral blood was positive for anti-HTLV-1 antibody, and Southern blots proved that HTLV-1 provirus DNA was present.

On the basis of these fi ndings, we diagnosed ATL in this patient and considered the possibility that the episcleritis was associated with the ATL. After topical betamethasone treatment was started, both the IOP and dilation of the episcleral vessels began to decrease (Fig. 2). On day 13 after admission, therapy with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) was begun because the dysfunction of the liver had progressed as a result of direct leukemic infi ltration. The day after CHOP therapy was

started, the dilation of the episcleral vessels and the IOP decreased further. After 1 week of CHOP therapy, both eyes were normal without any ophthalmic medications (Fig. 1c, d). Eventually, the patient’s general condition also markedly improved.

Comments

The ocular manifestations in this patient were bilateral and completely eliminated by topical corticosteroids and CHOP therapy, suggesting that they were associated with the ATL. Episcleritis and the elevation of the IOP were completely eliminated by topical corticosteroids. In the previous case report, episcleral biopsy showed infi ltration of only mono-nuclear cells, indicating that the episcleritis was related to the immune-mediated reaction induced by HTLV-1, and that the elevation of the IOP was due to the disturbance of venous perfusion by the infl ammation. As in a previously reported case,3 our patient recovered the ocular condition almost completely after topical betamethasone treatment was started. This suggests that, although a biopsy was not performed, the cause was an immune reaction mediated by HTLV-1.

In conclusion, ATL, especially the acute type, usually has a poor vital prognosis.4 However, early diagnosis of ATL, even of the acute type such as in this case, results in a good clinical course. Thus, ATL should be considered in patients with refractory conjunctivitis and bilateral elevated IOP of unknown causes. A relatively simple examination of the peripheral blood is useful in identifying ATL.

Key Words: adult T-cell leukemia, conjunctivitis, human T-cell lym-photrophic virus type-1, secondary glaucoma

Shinichirou Oono1, Takuji Kurimoto1, Hidehiro Oku2, and Osamu Mimura1

1 Department of Ophthalmology, Hyogo College of Medicine, Nishi-nomiya, Hyogo, Japan; 2 Department of Ophthalmology, Osaka Medical College, Takatsuki, Osaka, Japan

Received: February 5, 2008 / Accepted: July 31, 2008Correspondence to: Takuji Kurimoto, Department of Ophthalmology, Hyogo College of Medicine, 1-1 Mukogawa-cho, Nishinomiya, Hyogo 663-8501, Japane-mail: kurimoto@hyo-med.ac.jp

DOI 10.1007/s10384-008-0599-6

References

1. Ohba N, Matsumoto M, Sameshima M, et al. Ocular manifestations in patients infected with T-lymphotrophic virus type-1. Jpn J Oph-thalmol 1989;33:1–12.

2. Buggage RR. Ocular manifestations of human T-cell lymphotrophic virus type-1 injection. Curr Opin Ophthalmol 2003;14:420–425.

3. Goto K, Sugita M, Okada K, et al. Recurrent episcleritis associated with adult T-cell leukemia. Br J Ophthalmol 1993;77:743–744.

4. Yasunaga J, Matsuoka M. Human T-cell leukemia virus type 1 induces adult T-cell leukemia: From clinical aspects to molecular mechanisms. Cancer Control 2007;14:133–140.

Figure 2. Changes in the intraocular pressure (IOP) of both eyes after admission. After the initiation of topical betamethasone, the IOP gradually decreased bilaterally. In addition, after CHOP therapy (cyclophosphamide, doxorubicin, vincristine, and prednisone) com-bined with ophthalmic treatment, the IOP decreased to within normal range in both eyes. Nineteen days after admission, IOP was normalized by CHOP therapy even though ophthalmic treatment had ended. �, right eye; �, left eye.