A 55-year-old female had been referred to uveitis clinic of Khatam-Al-Anbia eye hospital for refractory, relapsing unilateral hypopyonic anterior uveitis (AU). She complained of decreased visual acuity in her right eye since 2 months ago associated with redness and ocular pain. The past medical, ocular, drug and familial history were unremarkable. At the first examination best corrected visual acuity (BCVA) was 20/32 and 20/20 in the right and left eye, respectively. Relative afferent pupillary defect was negative. Intraocular pressure was normal in both eyes. In slit lamp examination of the right eye, non-mutton fat keratic precipitates (KPs) and 0.2 mm hypopyon had been observed (Fig. 1a). Iris was bulged inferonasaly (Fig. 2a). Fundus examination and imaging were normal without any vitreous involvement (Fig. 2b, 4a). The left eye examination was normal (Fig. 3, 4b)
Regarding to hypopyonic AU, rheumatologic consult was done for excluding Behcet disease and ankylosing spondylitis and other rheumatologic diseases. Laboratory tests including complete blood count (CBC, diff), erythrocyte sedimentation rate (ESR), C-reactive protein(CRP), HLA B27, HLA B5, liver and kidney function tests, HIV and hepatitis serology, PPD test, peripheral blood smear and blood and urine culture for exclusion of endogenous endophthalmitis were performed. All tests and work ups got back normal. With the possible diagnosis of herpetic anterior uveitis, topical corticosteroid, cycloplegic and oral acyclovir treatment was prescribed without significant response.
Due to worsening of visual acuity (VA), persistence of anterior uveitis and iris bulging despite treatment with corticosteroid, systemic antiviral and antibiotics, anterior chamber (AC) sampling for polymerase chain reaction (PCR), cytologic and microbial evaluation was performed with suspicious of infectious and masquerades etiologies that were inconclusive. Systemic investigation for masquerade syndrome was also unremarkable. One month later, VA decreased to 2/100. In her examination 1 mm sero-sanguinous reaction was seen in AC and fundus was not visible. The left eye was normal again. There was no vitreous opacity in B-Scan of the right eye (Fig. 2b).
Again, with suspicion of intraocular lymphoma, we evaluated the patient for systemic lymphoma and leukemia. Brain and orbital magnetic resonance imaging (MRI), lumbar puncture and oncology consultation were requested. The results of all evaluations were negative. Second cytologic evaluation of AC sampling was negative for atypical cells, too.
Progressive decreasing VA to hand motion with increasing hypopyon without any response to anti-microbial treatment including virus, bacteria and fungi convinced us for third cytologic evaluation. Finally, cataract surgery without lens implantation associated with AC sampling for cytology and flow cytometry was performed with guidance of ocular pathologist. This sample confirmed large B cell lymphoma and it was positive for CD19 and CD20.
With the diagnosis of primary intraocular lymphoma which surprisingly affects only the anterior chamber, intravitreal methotrexate (MTX) 0.4 mg/0.1 cc weekly for one month and then monthly for 5 months and rituximab 1 mg/0.1 cc monthly for 2 month (regarding to the posterior capsule opening) were injected to the right eye. Significant response was observed after 2 weeks with disappearance of hypopyon, KPs and improvement of BCVA to 20/40 (Fig. 1c, 4c).
Six months after first presentation, second brain and orbital MRI revealed abnormal signal areas with increased enhancement (Fig. 5). In head and neck ultrasonography, 5 mm benign lymph node in her left axilla and 4 mm benign lymph node around right and left carotid artery was detected. After one month she complained of generalized tremor. According to hematologist and oncologist consult, systemic chemotherapy and radiotherapy was performed. In the last 18 months follow up, BCVA was 20/30 and the eye was quiet without posterior segment or fellow eye involvement with stable general condition (Fig. 1d).