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Characteristics of Ocular Adnexal Mucosa-associated Lymphoid Tissue Lymphoma after First-line Chemotherapy: a 7-year Experience
Ann Optom Contact Lens 2019;18:67-74
Published online September 25, 2019
© 2019 The Korean Optometry & Contact Lens Study Society

Sung Eun Kim, MD1*, Ji-Sun Paik, MD, PhD2*, Suk-Woo Yang, MD, PhD1

Department of Ophthalmology, Catholic University Lymphoma Group, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea1, Seoul, Korea
Department of Ophthalmology, Catholic University Lymphoma Group, Yeouido St. Mary's Hospital, College of Medicine, The Catholic University of Korea2, Seoul, Korea
Correspondence to: Suk-Woo Yang, MD, PhD
Department of Ophthalmology, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, #222 Banpo-daero, Seocho-gu, Seoul 06591, Korea
Tel: 82-2-2258-1188, Fax: 82-2-599-7405

* Sung Eun Kim and Ji-Sun Paik contributed equally to this work as first authors.
Received June 14, 2019; Revised August 22, 2019; Accepted September 6, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Purpose: Ocular adnexal mucosa-associated lymphoid tissue lymphoma (OAML) is the most common primary orbital malignancy. While radiotherapy is the treatment of choice for limited stage tumors, chemotherapy is required to treat disseminated disease or those patients who cannot tolerate radiotherapy. In this study, we examined the characteristics of OAML and treatment responses after first-line chemotherapy.
Methods: Eighty patients with histopathologically confirmed OAML were treated with a combination of cyclophosphamide, vincristine, and prednisolone (CVP) with selective addition of rituximab (R-CVP) or the combination of cyclophosphamide, hydroxydaunorubicin, oncovine and prednisolone (CHOP). Radiation therapy or additional chemotherapy with different regimens was undertaken for chemotherapy failure cases.
Results: The most common site of lymphoma was orbit and 25 (31.3%) patients had bilateral disease at presentation. The tumor stage was IE in 39 (48.8%) patients, IIE in 18 (22.5%) patients, IIIE in 3 (3.8%) patients, and IVE in 20 (25%) patients. Twenty-eight (35%) patients had no ocular symptoms and the common symptoms were foreign body sensation, periorbital swelling, and proptosis. Overall response rate of 100% was achieved in all patients regardless of chemotherapy regimen: complete remission (CR) was observed in 93.5% of patients treated with R-CVP, 66.7% with CHOP, and 72.7% with CVP. After a median follow-up of 54 months, relapse occurred in five (6.2%) patients and three of these patients underwent radiation therapy while the other two patients received additional chemotherapy. All patients except for two patients had CR as the final response.
Conclusions: First-line chemotherapy is effective and well tolerated in patients with localized (stage 1) and advanced stage (≥ stage 2) OAML. R-CVP therapy can achieve a higher treatment response than CHOP or CVP therapy, as well as higher progression-free survival.
Keywords : Chemotherapy; Marginal zone B cell lymphoma; Ocular adnexal mucosa-associated lymphoid tissue lymphoma

September 2019, 18 (3)