What Is the Treatment for Biopsy-Proven Conjunctival Extranodal Marginal Zone B-Cell Lymphoma with Bilateral Disease and No Systemic Involvement?

Conjunctival extranodal marginal zone B-cell lymphoma (EMZL) is a low-grade, localised form of ocular adnexal lymphoma. When histopathological biopsy confirms the diagnosis and staging reveals bilateral conjunctival disease with no systemic involvement, a structured first-line approach is indicated.

Clinical Scenario

Biopsy-proven conjunctival EMZL — bilateral conjunctival involvement, no systemic spread. Diagnosis is established by incisional biopsy for histopathological and cytological examination. Isolated conjunctival disease corresponds to localised staging (Ann Arbor stage I / AJCC T1–T2 N0M0), for which locoregional treatment is the primary consideration.

First-Line Treatment Approach

Established first-line options for this presentation include a radiation-based approach, a systemic agent, and an antibiotic-based strategy — with the choice guided by extent and laterality of disease. The full protocol specifies the regimens, sequences, and criteria for selecting among these approaches; only a partial overview is provided here.

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References

DOI: 10.1038/s41433-022-02176-2

The gold standard of conjunctival lymphoma diagnosis is an incisional biopsy for histopathological and cytological examination.
Radiotherapy is the gold standard treatment for lymphoma isolated to the conjunctiva, classified as Ann Arbor stage 1, or T1–T2 (N0M0) according to the AJCC criteria.
A dose range of 20–30 Gy was long advocated for management of these lesions, however recent literature suggests much lower doses can be used.
Fasola and colleagues were the first to apply this to OALs in 2013, whereby patients were administered 4 Gy delivered in two 2-Gy fractions on two consecutive days.
Nuckel and co-workers were the first to employ rituximab at an intravenous dose of 375 mg/m² weekly (for 4 weeks) in 2 patients with orbital disease, with a 50% complete response rate.
Doxycycline 100 mg twice daily has been shown to be a viable treatment option for T1N0M0 lymphoma with a 5-year progression free survival of 55%, where C. psittaci was confirmed.
There has been only one report to date describing 6 months of oral Clarithromycin (500 mg twice daily) in relapsed or refractory conjunctival EMZL.

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