Systemic Diffuse Large B-Cell Lymphoma with Bilateral Ciliary Body Involvement

Primary intraocular lymphoma (PIOL) is an exceedingly rare condition, with an estimated incidence of approximately 4.8 cases per million individuals annually in the United States, translating to roughly 300 new cases each year. In contrast, secondary intraocular lymphoma (SIOL) is even less common. SIOL typically arises when lymphoma cells spread to the eye from a distant site via the bloodstream, most often involving the uvea, particularly the choroid. Ciliary body involvement in SIOL is exceptionally rare, and cases of systemic diffuse large B-cell lymphoma (DLBCL) with bilateral ciliary body involvement are scarcely reported in the medical literature.

This report describes a 54-year-old male patient who presented to the Department of Ophthalmology at Peking Union Medical College Hospital on July 15, 2019, with complaints of redness, pain, and blurred vision in both eyes. Over the preceding three months, the patient had experienced progressively worsening vision, which had been diagnosed as bilateral anterior uveitis and secondary glaucoma. Initial treatment included 1% prednisolone eye drops, 2% carteolol hydrochloride eye drops, and 1% brinzolamide eye drops. Despite this treatment, the patient’s condition deteriorated, and he developed hypopyon and elevated intraocular pressure (IOP) three months later. At this point, he was referred for further evaluation.

The patient’s medical history revealed a prior diagnosis of systemic diffuse large B-cell lymphoma, confirmed through immunohistochemical analysis of an axillary lymph node biopsy. He had undergone six courses of chemotherapy, which included standard rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP regimen). Upon admission, his best-corrected visual acuity (BCVA) was 20/100 in the right eye and 20/200 in the left eye. Intraocular pressure was significantly elevated, measuring 42 mmHg in the right eye and 45 mmHg in the left eye (1 mmHg = 0.133 kPa).

Clinical examination revealed severe anterior chamber inflammation in both eyes, characterized by 3+ large keratic precipitates, 1+ flare, 4+ cells, and a pseudohypopyon in the inferior angle. The peripheral anterior chamber was obliterated bilaterally, and the iris appeared thickened with nodular areas of infiltration. Fundus examination was not possible due to the severity of the anterior segment involvement. B-scan ultrasound imaging demonstrated a clear vitreous cavity and the absence of retino-choroidal anomalies. Ultrasound biomicroscopy (UBM) revealed 360° thickening of the iris and ciliary body, along with peripheral angle closure.

To confirm the diagnosis, paracentesis of the anterior chamber and diagnostic vitrectomy were performed. Cytological analysis of the aqueous and vitreous specimens revealed atypical lymphoid cells, which were further characterized by gene rearrangement analysis as IgK and IgH positive. Brain magnetic resonance imaging (MRI) showed no significant abnormalities. Based on these findings, a diagnosis of secondary ciliary body lymphoma (diffuse large B-cell type) was established.

The patient was treated with intravitreal methotrexate (MTX) at a dose of 0.4 mg in 0.1 mL, administered twice weekly for four weeks, followed by weekly injections for an additional four weeks, and monthly injections for ten months. After the first MTX injection in each eye, the patient’s BCVA improved to 20/63 in the right eye and 20/100 in the left eye. Intraocular pressure decreased to 13 mmHg in the right eye and 19 mmHg in the left eye, and the pseudohypopyon resolved. The patient continued to receive systemic chemotherapy for his lymphoma.

Ciliary body involvement in lymphoma is typically associated with mild anterior inflammation. However, some cases, particularly those of SIOL, may present with more pronounced anterior segment reactions and keratic precipitates. In this patient, the intense anterior chamber inflammation and pseudohypopyon were consistent with previous reports of SIOL. Secondary glaucoma is also a common clinical finding in intraocular lymphoma cases, often resulting from dense tumor infiltration in the chamber angle, thickened iris, and ciliary body. In this case, the elevated IOP was likely due to these factors, and it was effectively controlled following intravitreal MTX treatment.

Ultrasound biomicroscopy (UBM) is a valuable diagnostic tool for detecting iris and ciliary body involvement in intraocular lymphoma. UBM provides high-resolution images of the anterior segment, allowing for precise visualization of tumor location and accurate measurements. In this patient, UBM revealed 360° thickening of the iris and ciliary body with acoustic solidity but low internal reflectivity, which helped differentiate the condition from uveitis.

The presence of tumor cells in the anterior uveal structures is crucial for diagnosing intraocular lymphoma. However, aqueous samples are often non-diagnostic due to low cellularity. In this case, the combination of paracentesis and diagnostic vitrectomy improved the diagnostic yield, enabling the identification of atypical lymphoid cells in both the aqueous and vitreous humor. Gene rearrangement analysis further confirmed the presence of diffuse large B-cell lymphoma.

In conclusion, ciliary body recurrence of lymphoma should be considered in patients with SIOL who present with pseudohypopyon and elevated IOP, particularly those with a history of systemic lymphoma. Vitreous or tissue biopsy remains the gold standard for diagnosing intraocular lymphoma. Treatment with chemotherapy or radiotherapy can help preserve visual acuity, and a multidisciplinary approach is essential for providing individualized care to patients with ciliary body lymphoma.

doi.org/10.1097/CM9.0000000000000580

Was this helpful?

YesNo

0 / 0