Ocular adnexal lymphomas (OAL) are malignant neoplasms which develop in the orbit, the conjunctiva and eyelid. The majority of lymphomas occurring in the ocular adnexa are represented by extranodal marginal zone B-cell lymphomas. In patients with stage IE OAL, localized radiotherapy is the therapy of choice. Complications, however, are frequent, including dryness of the eye, corneal ulcerations, cataract and retina damage. Surgical treatment is usually recommended for conjunctival and eyelids lymphomas but fails to control the growth of lymphoma. Contralateral eye or sistemic relapses are reported in about 10–25% of cases. Therefore, a systemic approach is needed. Rituximab is an anti-CD20 monoclonal antibody, and its efficacy has been demonstrated in indolent and aggressive lymphomas in combination with chemotherapy. Since December 2003 to January 2006 we treated 9 patients with OAL with a combination of Rituximab and Chlorambucil according to this schedule: four weekly infusions of Rituximab 375 mg/m2 plus Chlorambucil 0,1 mg/Kg/die for 45 days, followed by Rituximab 375 mg/m2 once a month for four months and Chlorambucil 0,1 mg/Kg/die for two weeks every month. Eight pts had a diagnosis of extranodal marginal zone B-cell lymphoma and one patient a follicular grade I lymphoma. Median age at diagnosis was 78 years (range 56–86). All pts had a stage I disease and no pts presented systemic symptoms. Disease was localized in the conjunctiva in 6 pts (4 right and 2 left), in two pts the disease was observed in the orbit and in one patient it was localized in the eyelid. IPI was evaluable in 7 pts, and all had a low risk. All pts completed the program without delays. Toxicity, both hematological and extrahematological, was mild, in spite of the high median age of the pts. At the end of treatment 8 pts obtained a complete remission (CR), and a patient obtained a partial response. After a median follow-up of 17 months all pts are alive, and 8 out of 9 (89%) are still in CR. No late toxicity of the ocular adnexa was observed. In conclusion, we hypothesize that Rituximab in combination with Chlorambucil should be take into consideration as first line treatment of primary ocular adnexa lymphomas due to its feasibility and the lacking of severe toxicity and local sequelae.

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