Erythematous patches and plaques involving the eyelids from mycosis fungoides (A), and dense lobular hypopyon mixed with blood in the right (B) and left (C) eyes.
Cytological analysis. A, Smear of aqueous humor in the left eye with May, Grunwald, and Giemsa stains, showing enlarged, pleomorphic lymphocytes with nuclear clefts (arrows), convolutions, and multiple nucleolar organizing regions and mitotic figures (original magnification ×1200). Top right inset, Positive immunocytological staining of the lymphocytes for CD3 (anti-CD3 stain, original magnification ×400). Top left inset, Nucleolar organizing regions (May, Grunwald, and Giemsa stains, original magnification ×1200). Bottom right inset, A mitotic figure (May, Grunwald, and Giemsa stains, original magnification ×1200). Resolution of hypopyon occurred in the right (B) and left (C) eyes after radiation to bilateral orbits and the brain.
Ralli M, Goldman JW, Lee E, Pinter-Brown LC, Glasgow BJ, Sarraf D. Intraocular Involvement of Mycosis Fungoides. Arch Ophthalmol. 2009;127(3):343-345. doi:10.1001/archophthalmol.2009.6
Mycosis fungoides (MF) is a malignant cutaneous T-cell lymphoma characterized by erythematous patches, plaques, and tumors. In later stages, noncutaneous involvement can ensue with visceral spread, lymphadenopathy, and Sézary syndrome.1 Intraocular extension is rare,2 and anterior chamber involvement has not been reported. We describe a case of bilateral hypopyon from intraocular MF involvement.
A 68-year-old man with MF, stage T4N3M1B0 by TNMB classification,3 complicated by inguinal lymphadenopathy and epiglottal involvement had worsening vision over 2 weeks. He had previously been treated with UV light therapy enhanced with psoralen, gemcitabine hydrochloride, interferon, methotrexate sodium, and liposomal doxorubicin hydrochloride. Visual acuity was 20/25 OD and hand motions OS. His skin was diffusely hyperemic and edematous with several tumors (Figure 1A). Dense, bilateral, lobular-appearing hypopyon with admixed blood was present in each eye. Both irides had prominent neovascularization with rugate appearances and dense posterior synechiae (Figure 1B and C). B-scan ultrasonography showed no significant vitreous opacification or retinochoroidal infiltration, and ultrasound biomicroscopy revealed bilaterally thickened iris roots.
Biopsies of the aqueous humor and vitreous as well as intravitreous injection of vancomycin hydrochloride, ceftazidime, and dexamethasone were performed in the left eye. Vitreous cultures were negative, but aqueous humor smears showed large, pleomorphic lymphocytes with irregular, convoluted nuclei and nuclear clefts. Immunocytological staining was positive for CD3 (Figure 2A) and negative for CD20. Monoclonal T-cell gene rearrangement of the T-cell receptor γ locus was identified by polymerase chain reaction. Peripheral blood smear and flow cytometry results were within normal limits. Magnetic resonance imaging of the brain showed right optic nerve sheath enhancement. The patient was treated with 30 Gy of external beam irradiation in 10 fractions (to convert gray to rad, multiply by 100) to the orbits and brain with complete resolution of hypopyon in both eyes on day 10 of therapy with visual acuities of 20/25 OD and 20/60 OS (Figure 2B and C). He declined further treatment.
The malignant cell in MF, distinguished by its hyperconvoluted nucleus, derives from mature CD4 postthymic lymphocytes with a propensity to home to the epidermis. Sézary syndrome is a variant of MF, characterized by the presence of these malignant cells in the peripheral circulation.1
Ophthalmic manifestations of MF are typically associated with advanced disease. Cook et al2 followed up 42 cases of ophthalmic involvement of cutaneous T-cell lymphoma, 31 of which had either MF or Sézary syndrome. The eyelids were most frequently involved; corneal, scleral, and optic nerve manifestations were more rarely observed. Two cases of retinal infiltration were noted. Other reports4,5 have described vitritis and choroidal infiltration, and one study5 found neoplastic cells between the Bruch membrane and the retinal pigment epithelium in an autopsy specimen from a patient with MF.
Contrary to these previous reports of intraocular MF, our patient had no apparent vitreoretinal disease but did have iris infiltration and resultant bilateral hypopyon in addition to radiologically presumed right optic nerve sheath involvement. Anterior chamber MF involvement has not been previously reported to our knowledge. Our patient had documented lymphatic spread but lacked laboratory evidence of hematologic dissemination. Given the absence of ocular lymphatics, this case in conjunction with other reports of intraocular MF suggests that hematologic spread of MF can occur despite normal hematologic laboratory evaluation results. Seeding could occur through the anastomotic connections between the palpebral and anterior ciliary venous circulations. Alternatively, lymphatic drainage via the thoracic duct may seed the ciliary artery distribution.
Although our patient responded to radiotherapy with hypopyon resolution and improved vision, intraocular involvement of cutaneous T-cell lymphomas historically portends a poor prognosis, with reported survival of 17 days to 1 year after diagnosis.4- 6
Given the immunocompromised state that malignant neoplasms and chemotherapy can confer, suspicion for infection in these patients with hypopyon is necessarily high. However, as demonstrated here, intraocular malignant neoplasms must also be considered and biopsy of the aqueous and/or vitreous is prudent.
Correspondence: Dr Sarraf, Jules Stein Eye Institute, University of California, Los Angeles, 100 Stein Plaza, Los Angeles, CA 90095 (firstname.lastname@example.org).
Financial Disclosure: None reported.
Funding/Support: This work was supported in part by a grant from Research to Prevent Blindness (Dr Sarraf).