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Abstract
Purpose
To report the effectiveness of rituximab, cyclophosphamide, vincristine, and prednisolone (R-CVP) combination chemotherapy in patients with ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (OAML).
Methods
R-CVP chemotherapy was performed in 7 patients (8 eyes) with a confirmed histopathological diagnosis of OAML from January 2012 to December 2012. A total of 6 cycles were administered at a 3-4 week interval and 2 cycles of rituximab were added at the third week. Response evaluation was performed 3 times, with the first evaluation 3 weeks after the third cycle, the second 3 weeks after the sixth cycle, and the last after the second rituximab cycle.
Results
In all 7 patients (8 eyes) with R-CVP, symptoms were improved, and the mass was markedly resolved based on orbit CT and MRI scan at first response evaluation. Clinically complete remission (CR) was achieved in 6 patients and par-tial response (PR) in 1 patient. At final response assessment, there was no detectable mass on orbit CT or MRI. There were no severe infections or hematologic adverse effects including neutropenia or decreased immunoglobulin during the follow-up period.
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 | Figure 1.Right eye shows upper lid swelling, proptosis, conjunctival injection and chemosis (A, B). Upper lid swelling, proptosis, conjunctival injection and chemosis, medial gaze limitation in right eye have markedly decreased after R-CVP infusion (C, D). |
 | Figure 2.Coronal MRI scans show enhancing mass in medial aspect (involving conal/intraconal/extraconal spaces) of right orbit (postseptal location). (A) It extends to orbital apex, but there is no evidence of tumor invasion into right optic canal or intracranial extension. After R-CVP infusion, size and enhancement of known orbital MALT lymphoma in right postseptal medial area have moderately decreased. (B) In CT scans no enhancing mass was detected (figure not shown). |
 | Figure 3.Right eye shows proptosis, conjunctival injection and chemosis. Down and lateral gaze limitation of right eye is prominent (A, B). Proptosis, conjunctival injection and chemosis, down and lateral gaze limitation in right eye have markedly decreased after R-CVP infusion (C, D). |
 | Figure 4.Coronal CT scans show sharply defined high density mass with moderate homogenous enhancement in retro-bulbar space of right orbit (A). No evidence of adjacent bony erosion or intracranial extension. After R-CVP infusion, markedly decreased extent of homogenous enhancing mass in retrobulbar space of right orbit has seen (B). |
 | Figure 5.Both eyes show lower conjunctival injection and salmon patch appearance mass-like lesion (A, C). Lower conjunctival in-jection and chemosis in both eye have markedly decreased after R-CVP infusion. In CT and MRI scans, no visualization of discrete mass lesion in both orbits and periorbital (B, D). |
Table 1.
Demographics and clinical characteristics in patients treated with R-CVP diagnosed with ocular adnexal MALT lymphoma
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