Blood Res 2018; 53(4): 307-313  https://doi.org/10.5045/br.2018.53.4.307
Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand
Kasem Seresirikachorn1, Lalita Norasetthada2, Sakarin Ausayakhun1, Atitaya Apivatthakakul1, Sirima Tangchittam3, Vannakorn Pruksakorn3, Kitsada Wudhikarn4, Damrong Wiwatwongwana1
1Department of Ophthalmology, 2Department of Medicine, Division of Hematology, Faculty of Medicine, Chiang Mai University, Chiang Mai, 3Department of Ophthalmology, 4Department of Medicine, Division of Hematology, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand
Correspondence to: Lalita Norasetthada, M.D., Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand, E-mail: Lalita.n@cmu.ac.th
Received: August 2, 2018; Revised: August 13, 2018; Accepted: August 27, 2018; Published online: December 31, 2018.
© The Korean Journal of Hematology. All rights reserved.

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Abstract
Background
Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand.
Methods
We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients’ clinical characteristics and treatment outcomes.
Results
Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22‒86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01).
Conclusion
We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.
Keywords: Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, Ocular adnexal lymphoma, MALT lymphoma, Radiotherapy


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