Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand

Kasem Seresirikachorn; Lalita Norasetthada; Sakarin Ausayakhun; Atitaya Apivatthakakul; Sirima Tangchittam; Vannakorn Pruksakorn; Kitsada Wudhikarn; Damrong Wiwatwongwana

doi:10.5045/br.2018.53.4.307

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Original Article

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Blood Res 2018; 53(4):

Published online December 31, 2018

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 Norasetthada^2*, Sakarin Ausayakhun¹, Atitaya Apivatthakakul¹, Sirima Tangchittam³, Vannakorn Pruksakorn³, Kitsada Wudhikarn⁴, and Damrong Wiwatwongwana¹

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¹Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

²Division of Hematology, Department of Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

³Department of Ophthalmology, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

⁴Division of Hematology, Department of Medicine, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

Correspondence to : Correspondence to Lalita Norasetthada, M.D. Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand, Lalita.n@cmu.ac.th

Received: August 2, 2018; Revised: August 13, 2018; Accepted: August 27, 2018

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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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

Article

Original Article

Blood Res 2018; 53(4): 307-313

Published online December 31, 2018 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 Norasetthada^2*, Sakarin Ausayakhun¹, Atitaya Apivatthakakul¹, Sirima Tangchittam³, Vannakorn Pruksakorn³, Kitsada Wudhikarn⁴, and Damrong Wiwatwongwana¹

¹Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

²Division of Hematology, Department of Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand.

³Department of Ophthalmology, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

⁴Division of Hematology, Department of Medicine, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.

Correspondence to:Correspondence to Lalita Norasetthada, M.D. Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Chiang Mai University, Chiang Mai 50200, Thailand, Lalita.n@cmu.ac.th

Received: August 2, 2018; Revised: August 13, 2018; Accepted: August 27, 2018

Abstract

Background

Methods

Results

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

Abstract

Fig 1.

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Figure 1.

Kaplan-Meier curve of progression-free survival (PFS) in patients with primary ocular adnexal MALT lymphoma (A) and primary ocular adnexal MALT lymphoma (B).

Blood Research 2018; 53: 307-313https://doi.org/10.5045/br.2018.53.4.307

Fig 2.

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Figure 2.

Kaplan-Meier curve of progression-free survival (PFS) in patients with primary ocular adnexal MALT lymphoma, according to Ann Arbor stage. (A) Five-year PFS 77.4% versus 52.5% for patients with limited- versus advanced-stage disease (P=0.063). (B) Five-year OS 96.0% versus 81.7% for patients with limited- versus advanced-stage disease (P=0.123).

Blood Research 2018; 53: 307-313https://doi.org/10.5045/br.2018.53.4.307

Fig 3.

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Figure 3.

(A) Kaplan-Meier progression-free survival (PFS) between radiotherapy and non-radiotherapy-based treatment in patients with limited-stage primary ocular adnexal MALT lymphoma. Five-year PFS 89.9% and 52.9%, respectively (P=0.007). (B) Kaplan-Meier progression-free survival (PFS) comparison between radiotherapy-containing therapy and non-radiotherapy-containing therapy in patients with advanced-stage primary ocular adnexal MALT lymphoma. Five-year PFS 66.7% and 49.5%, respectively (P=0.52).

Blood Research 2018; 53: 307-313https://doi.org/10.5045/br.2018.53.4.307

Table 1 Demographic and clinical characteristics of primary POML.

Abbreviations: ECOG, Eastern Cooperative Oncology Group; HBV, hepatitis B virus; HCV, hepatitis C virus; HIV, human immunodeficiency virus; LDH, lactate dehydrogenase; POML, primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue..

Table 2 Treatment modalities, response rates, and relapse, according to staging.

Abbreviations: CR, complete response; OR, odds ratio; PR, progression; RT, radiotherapy..

Table 3 Selected large cohort studies investigating POML.

Abbreviations: CT, chemotherapy; EFS, event-free survival; N, number of cases; NA, not applicable; OS, overall survival; PFS, progression-free survival; POML, primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue lymphoma; RT, radiotherapy..