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Primary Ciliary Body Marginal Zone Lymphoma Presenting as Hemorrhagic Hypopyon
İrem Koç1, Hayyam Kiratli1, Yasemin Kapucu1
1Department of Ophthalmology, Ocular Oncology Service, Ankara, Turkey.
Insights
Primary ciliary body lymphoma, a rare cause of persistent anterior uveitis, was diagnosed in a patient presenting with hemorrhagic hypopyon. Early diagnosis and biopsy are crucial for effective treatment of this extranodal marginal zone lymphoma.
Area of Science:
- Ophthalmology
- Oncology
- Hematology
Background:
- Extranodal marginal zone lymphoma (EMZL) can rarely involve the ciliary body.
- Ciliary body lymphoma may present as refractory anterior uveitis.
Observation:
- A 59-year-old man presented with unilateral anterior uveitis and hemorrhagic hypopyon unresponsive to treatment.
- Anterior segment ultrasound biomicroscopy identified an iridociliary mass.
- Diagnostic cyclectomy revealed neoplastic cells positive for CD20, lambda light chain, and BCL 2.
Findings:
- Histopathological evaluation confirmed extranodal marginal zone lymphoma of the ciliary body.
- Immunohistochemistry excluded other lymphoid malignancies based on specific marker negativity.
Implications:
- Ciliary body lymphoma should be considered in cases of intractable anterior uveitis.
- Repeat biopsies may be necessary for definitive diagnosis when clinical suspicion is high.
Purpose:
Our purpose is to report a patient with primary unilateral ciliary body marginal zone lymphoma who initially presented with hemorrhagic hypopyon.
Methods:
Retrospective review of the clinical, imaging, and immunohistopathological features of the case was performed.
Results:
A 59-year-old man was referred with right anterior uveitis of unknown etiology which was unresponsive to systemic treatment. Slit-lamp biomicroscopy showed normotensive hemorrhagic hypopyon in that eye. Anterior segment ultrasound biomicroscopy revealed an iridociliary mass lesion. Because an anterior chamber paracentesis was noncontributory, a diagnostic cyclectomy was performed. Histopathological evaluation showed that the neoplastic cells were positive for CD20, lambda light chain, and BCL 2. BCL 6, CD10, CD5, SOX11, kappa, and Cyclin D1 stains were negative. The final diagnosis was extranodal marginal zone lymphoma of the ciliary body.
Conclusions:
Although rare, ciliary lymphoma may be a cause of intractable anterior uveitis. Repeat biopsies could be carried out when there is a high level of clinical suspicion.
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