One Chinese case with benign reactive lymphoid hyperplasia of the uvea.

Sir,

Benign reactive lymphoid hyperplasia (BRLH) of the uvea is usually considered as inflammatory pseudotumors of the uvea or intraocular pseudotumor.[1] It typically affects the middle-aged individuals and usually is unilateral. All reported cases were Caucasian. No apparent sexual predilection is observed. This disease is characterized by subretinal solitary or multiple creamy yellowish patches with diffuse choroidal thickening and retinal detachment. Differential diagnoses of this disease include diffuse malignant melanoma, metastatic carcinoma, systemic lymphoma, posterior scleritis, sarcoidosis, and infectious causes.[12]

A 49-year-old female patient was referred with left eye painless vision decline. Fundus examination showed radiate puckers in the posterior and macular retina [Fig. 1]. B-scan showed thickening of the choroid and sclera. She was diagnosed as scleritis and had been treated with 5 mg dexamethasone periocular injection. One year after that, she had the relapse with red eye, pain, and vision decline. Slit lamp examination showed mild active anterior chamber inflammation. Fundus examination revealed slight vitreous haze, multiple creamy yellowish patches, and serous retinal detachment. She had no system disease history. Purified protein derivative (PPD) skin test was negative. Computed tomography of the orbit, chest, and abdomen showed no abnormality. Magnetic resonance imaging of the brain did not disclose lymphoma. She had been treated with corticosteroids and cycloplegic eye drops. Dexamethasone was injected periocularly. Retinal detachment disappeared and yellow lesion was same as before. She suddenly had severe eye pain and vision decline to light perception one month after phacoemulsification and intraocular lens implantation. Ultrasound showed diffuse thickening of the choroid and sclera and severe retinal detachment [Fig. 2]. She had been treated with prednisone (1 mg/kg day) orally. Eye pain and retinal detachment disappeared gradually [Fig. 3], but vision declined to no light perception. After withdrawal of prednisone, eye pain occurred again. She refused corticosteroids treatment because of side effect and selected enucleation.

Figure 1

Fundus image at the onset

Figure 2

Ultrasound showed diffuse thickening of the choroid and sclera and severe retinal detachment

Figure 3

Fundus image after treatment with prednisone

Histopathologic examination showed a large number of lymphoid follicles with germinal centers in the choroid but not in iris and ciliary body. Extraocular extension of the lymphocyte infiltrates involved sclera and rectus muscle. Immunohistochemistry showed that T-cells predominantly located in the interfollicular zones and percolated throughout the center of the follicles, and B-cells mainly located in the center of follicles. Moreover, no prominent spillover was observed. Proliferation rate determined by Ki-67 immunostaining was about 2%, with heavy staining of lymphoid follicles with germinal centers [Fig. 4]. In situ hybridization with antibody to Bcl-2, Bcl-6, kappa, and lambda also revealed the benign feature.

Figure 4

Immunohistochemical characterization (left) CD3 stains T-cells in the interfollicular zones, (right) CD20 stains B-cells within the follicles

This case suggests that BRLH of the uvea should be taken into consideration in non-Caucasian patients with creamy yellow patches, especially when all the examinations of the brain and chest are negative. Chorioretinal biopsy could provide the definite evidence for the diagnosis.[3] It was reported that prednisone and radiation treatment were effective in controlling the inflammation of RLH.[4] However, in this case, inflammation recurred after the withdrawal of prednisone. It maybe suggests that the time to use prednisone is crucial. Furthermore, whether the immunosuppressive agents could control this disease still needs to be studied.

Financial support and sponsorship

The International Postdoctoral Exchange Fellowship Program 2014 by the Office of China Postdoctoral Council (20140038).

Conflicts of interest

There are no conflicts of interest.