First-Line Treatment of Biopsy-Proven Conjunctival Extranodal Marginal Zone B-Cell Lymphoma with Unilateral Disease and No Systemic Involvement
This protocol addresses the management of biopsy-confirmed conjunctival extranodal marginal zone B-cell lymphoma (EMZL) confined to a single eye, with no evidence of systemic lymphoma. This localised presentation allows for targeted, eye-directed first-line treatment.
Clinical scenario
Histopathological diagnosis of conjunctival EMZL is established by incisional biopsy. Disease is unilateral and localised to the conjunctiva, with systemic staging confirming no nodal or distant involvement. In elderly or frail patients with minimal symptoms, a monitored observation approach may be considered, particularly given the indolent nature of unilateral disease.
Treatment approach (partial overview)
First-line options for this localised presentation include intralesional immunomodulatory therapy administered locally to the eye, or external beam radiation therapy to the affected area. Surgical excision may be appropriate in select, well-circumscribed cases. The full structured protocol specifies the approach, sequencing, and criteria for each option.
Complete regimen details, eligibility criteria, and decision algorithm available via the structured protocol below.
References
DOI: 10.1038/s41433-022-02176-2
- The gold standard of conjunctival lymphoma diagnosis is an incisional biopsy for histopathological and cytological examination.
- A "wait and watch" strategy has been proposed for elderly or frail patients with little symptoms, particularly in the setting of unilateral disease.
- Subconjunctival instillation of 1–1.5 million international units (IU) of Interferon in 0.25 mL is advocated—with treatment frequencies up to 3 times weekly for 4–6 weeks, with further cycles advocated in the presence of incomplete response.
- An alternative dosing was proposed by Ferrari et al. who administered 1 to 2 mL of undiluted rituximab (10–20 mg/mL) plus 1 mL of 2% xylocaine.
- 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.
- In some cases, excisional biopsy or surgical resection may serve as therapy for well circumscribed conjunctival EMZL, however, in most cases attempts for complete resection should not be made because this does not influence survival and can lead to recurrence.
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