Treatment of Biopsy-Proven Bilateral Conjunctival Extranodal Marginal Zone B-Cell Lymphoma Without Systemic Involvement

This protocol covers the management of conjunctival extranodal marginal zone B-cell lymphoma (EMZL) confirmed by biopsy, presenting bilaterally in the conjunctiva with no evidence of systemic disease.

Clinical Scenario

Biopsy-proven conjunctival EMZL with bilateral conjunctival disease and no systemic involvement. The diagnosis rests on histopathological and cytological examination from an incisional biopsy specimen.

Treatment Approach

For bilateral conjunctival EMZL without systemic spread, the protocol incorporates systemic chemotherapy — both single-agent and combination regimens are among the options addressed.

Full regimen selection, decision criteria, and sequencing are detailed in the complete structured protocol…

Instant Access to Structured Evidence-Based Regimens

References

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.

Further, in patients with high grade localised disease, systemic chemotherapy may also be considered if there are contraindications to, intolerance or poor response to first line EBRT.

The standard single-agent chemotherapy for non-Hodgkin’s lymphoma remains chlorambucil, whereas combination therapy used includes cyclophosphamide, vincristine and prednisone (CVP), and cyclophosphamide, hydroxydaunorubicin, vincristine and prednisone (CHOP).

DOI: 10.1038/s41433-022-02176-2 View source ↗