Journal List > J Korean Ophthalmol Soc > v.54(8) > 1009439

J Korean Ophthalmol Soc. 2013 Aug;54(8):1157-1164. Korean.
Published online August 14, 2013.
Copyright © 2013 The Korean Ophthalmological Society
The Effect of Rituximab, Cyclophosphamide, Vincristine, and Prednisolone (R-CVP) Chemotherapy in Patients with Ocular Adnexal Extranodal Marginal Zone B Cell Lymphoma of the Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma
Sung Jin Park, MD,1 Won Sik Lee, MD, PhD,3 and Jae Wook Yang, MD, PhD1,2
1Department of Ophthalmology, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.
2Ocular Neovascular Disease Research Center, Busan, Korea.
3Department of Internal Medicine, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.

Address reprint requests to Jae Wook Yang, MD, PhD. Department of Ophthalmology, Busan Paik Hospital, #75 Bokji-ro, Busanjin-gu, Busan 614-735, Korea. Tel: 82-51-890-8710, Fax: 82-51-890-6329, Email:
Received December 29, 2012; Revised March 26, 2013; Accepted June 25, 2013.



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).


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.


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 partial 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.


In OAML, R-CVP combination chemotherapy is considered an effective, safe and important therapeutic approach, reducing the limitations of classic localized radiotherapy and combination chemotherapy and improving the remission rate.

Keywords: Ocular Adnexal MALT Lymphoma; Radiotherapy; R-CVP


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).
Click for larger image

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).
Click for larger image

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).
Click for larger image

Figure 4
Coronal CT scans show sharply defined high density mass with moderate homogenous enhancement in retrobulbar 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).
Click for larger image

Figure 5
Both eyes show lower conjunctival injection and salmon patch appearance mass-like lesion (A, C). Lower conjunctival injection 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).
Click for larger image


Table 1
Demographics and clinical characteristics in patients treated with R-CVP diagnosed with ocular adnexal MALT lymphoma
Click for larger image


This study was presented as a narration at the 108th Annual Meeting of the Korean Ophthalmology Society 2012.

This study was supported by a grant from the Korea Healthcare Technology R&D Project, Ministry of Health and Welfare Affairs, Republic of Korea (Grant NO. HI12C0005).

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