Blood Res 2019; 54(1):
Published online March 31, 2019
https://doi.org/10.5045/br.2019.54.1.4
© The Korean Society of Hematology
1Department of Internal Medicine, Dong-A University Hospital, Busan, Korea.
2Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
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.
The optimal management of marginal zone lymphoma (MZL) has yet to be clearly determined. Antibiotics, surgery, radiation, immunotherapy, and chemotherapy, either alone or in combination, have been previously employed in clinical practice, as well as watchful waiting. It has been well established that localized stage I/II MZL can be properly controlled with local modalities - namely, radiotherapy and/or surgery. A previous retrospective observational study [1] showed 5-year progression-free survival (PFS) and overall survival (OS) rates of 74.7% and 95.9%, respectively. In a later study, the benefit of adding chemotherapy to improve radiotherapy efficacy was not clear [2]. Considering these findings, local treatment should be considered the principal treatment modality for stage I/II MZL. Considering the indolent natural history of MZL, less toxic chemotherapeutic or immunotherapeutic agents are generally preferred. A prospective rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) clinical trial was conducted as a first-line treatment for advanced stage MZL [3]. There were 24 complete responses (CR) (60%), 11 partial responses (PR) (27.5%), four with stable disease (SD) (10%), and one with progressive disease (PD) (2.5%), yielding a response rate of 87.5% (95% CI, 77.1–97.9%). The estimated three-year PFS and OS were 59.5% and 95.0%, respectively. A more recent retrospective study showed that advanced-stage MZL patients treated with R-CVP had a 3-year PFS rate of 69.6% [4].
According to a retrospective multicenter analysis of 67
The same study also analyzed a total of 29
Localized stage OA-MZL can be controlled fairly effectively via radiotherapy [6]. A previous study demonstrated that localized stage OA-MZL can be controlled quite effectively with low-dose radiation, and its effects can persist for a long duration (more than 5 yr). Even bilateral synchronously involved OA-MZL can achieve 80.9% CR and 16.7% PR with radiotherapy of 27 Gy (range, 20–40 Gy) delivered to each eye [7].
The use of doxycycline for the eradication of
Despite the effective local control of tumor, radiotherapy has the disadvantages of ophthalmologic toxic effects, including late complications such as radiation cataract, xerophthalmia, ischemic retinopathy, glaucoma, and corneal ulceration. In a Korean prospective phase II trial [9], 33 patients with Ann Arbor stage I OA-MZL with the adverse factors were enrolled. They received six cycles of R-CVP followed by two cycles of rituximab therapy. The cumulative CR achievement was 93.9% at 2 years. PFS and OS at 4 years was 90.3% and 100%, respectively. R-CVP could be an alternative frontline therapy for limited-stage OA-MZL patients with adverse prognostic factors.
The optimal management of P-MZL lymphoma has yet to be clearly determined. Options include watchful waiting, or surgery, chemotherapy, and radiation therapy alone or in combination. Advanced or disseminated P-MZL involving both lungs or extra-pulmonary sites could be controlled via chemotherapy. In the lung, even with localized lesions, radiation and surgical excision of segments or lobes should be carefully considered due to risk of surgical complications, reduction in pulmonary function, and the generally favorable clinical course of MZL itself. In a Korean study that investigated prognosis and optimal approach to P-MZL patients [10], 56 of 61 total patients were treated with surgery (N=22), chemotherapy (12 CVP, 9 R-CVP, 4 CHOP, and 2 R-CHOP) (N=28), or radiotherapy (N=6). Forty-six patients (82.1%) achieved CR or PR. The median PFS was 5.6 years (95% CI, 2.6–8.6). There was no significant difference in PFS between chemotherapy and surgery (
The most frequently observed I-MZL involvement site was the ileo-cecal region (40.7%). Advanced-stage I-MZL cases were observed at a higher rate than in MZL of other sites. Musshoff's stage IE, IIE1, IIE2, IIIE, and IV were present in 44%, 15%, 11%, 7.4%, and 22%, respectively. Considering the clinical features of MZLs, local treatment can be regarded as a principal treatment modality. A high rate of CR was achieved with treatments including local modalities. Even in advanced stages of the disease, surgical treatment was employed in most of the patients (62.5%). This is because almost all patients suffered from subjective symptoms, and the small intestine and ileo-cecal region are difficult regions in which to perform endoscopic biopsy for tissue diagnosis. CR and PR were achieved in 82% and 4% patients, respectively. The estimated 5-year OS and PFS rates were 86% and 54%, respectively. Regardless of stage, I-MZL was controlled relatively well with combined treatment.
Blood Res 2019; 54(1): 4-6
Published online March 31, 2019 https://doi.org/10.5045/br.2019.54.1.4
Copyright © The Korean Society of Hematology.
Sung Yong Oh, M.D. Ph.D.1, and Cheolwon Suh, M.D. Ph.D.2
1Department of Internal Medicine, Dong-A University Hospital, Busan, Korea.
2Department of Oncology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
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.
The optimal management of marginal zone lymphoma (MZL) has yet to be clearly determined. Antibiotics, surgery, radiation, immunotherapy, and chemotherapy, either alone or in combination, have been previously employed in clinical practice, as well as watchful waiting. It has been well established that localized stage I/II MZL can be properly controlled with local modalities - namely, radiotherapy and/or surgery. A previous retrospective observational study [1] showed 5-year progression-free survival (PFS) and overall survival (OS) rates of 74.7% and 95.9%, respectively. In a later study, the benefit of adding chemotherapy to improve radiotherapy efficacy was not clear [2]. Considering these findings, local treatment should be considered the principal treatment modality for stage I/II MZL. Considering the indolent natural history of MZL, less toxic chemotherapeutic or immunotherapeutic agents are generally preferred. A prospective rituximab, cyclophosphamide, vincristine, and prednisone (R-CVP) clinical trial was conducted as a first-line treatment for advanced stage MZL [3]. There were 24 complete responses (CR) (60%), 11 partial responses (PR) (27.5%), four with stable disease (SD) (10%), and one with progressive disease (PD) (2.5%), yielding a response rate of 87.5% (95% CI, 77.1–97.9%). The estimated three-year PFS and OS were 59.5% and 95.0%, respectively. A more recent retrospective study showed that advanced-stage MZL patients treated with R-CVP had a 3-year PFS rate of 69.6% [4].
According to a retrospective multicenter analysis of 67
The same study also analyzed a total of 29
Localized stage OA-MZL can be controlled fairly effectively via radiotherapy [6]. A previous study demonstrated that localized stage OA-MZL can be controlled quite effectively with low-dose radiation, and its effects can persist for a long duration (more than 5 yr). Even bilateral synchronously involved OA-MZL can achieve 80.9% CR and 16.7% PR with radiotherapy of 27 Gy (range, 20–40 Gy) delivered to each eye [7].
The use of doxycycline for the eradication of
Despite the effective local control of tumor, radiotherapy has the disadvantages of ophthalmologic toxic effects, including late complications such as radiation cataract, xerophthalmia, ischemic retinopathy, glaucoma, and corneal ulceration. In a Korean prospective phase II trial [9], 33 patients with Ann Arbor stage I OA-MZL with the adverse factors were enrolled. They received six cycles of R-CVP followed by two cycles of rituximab therapy. The cumulative CR achievement was 93.9% at 2 years. PFS and OS at 4 years was 90.3% and 100%, respectively. R-CVP could be an alternative frontline therapy for limited-stage OA-MZL patients with adverse prognostic factors.
The optimal management of P-MZL lymphoma has yet to be clearly determined. Options include watchful waiting, or surgery, chemotherapy, and radiation therapy alone or in combination. Advanced or disseminated P-MZL involving both lungs or extra-pulmonary sites could be controlled via chemotherapy. In the lung, even with localized lesions, radiation and surgical excision of segments or lobes should be carefully considered due to risk of surgical complications, reduction in pulmonary function, and the generally favorable clinical course of MZL itself. In a Korean study that investigated prognosis and optimal approach to P-MZL patients [10], 56 of 61 total patients were treated with surgery (N=22), chemotherapy (12 CVP, 9 R-CVP, 4 CHOP, and 2 R-CHOP) (N=28), or radiotherapy (N=6). Forty-six patients (82.1%) achieved CR or PR. The median PFS was 5.6 years (95% CI, 2.6–8.6). There was no significant difference in PFS between chemotherapy and surgery (
The most frequently observed I-MZL involvement site was the ileo-cecal region (40.7%). Advanced-stage I-MZL cases were observed at a higher rate than in MZL of other sites. Musshoff's stage IE, IIE1, IIE2, IIIE, and IV were present in 44%, 15%, 11%, 7.4%, and 22%, respectively. Considering the clinical features of MZLs, local treatment can be regarded as a principal treatment modality. A high rate of CR was achieved with treatments including local modalities. Even in advanced stages of the disease, surgical treatment was employed in most of the patients (62.5%). This is because almost all patients suffered from subjective symptoms, and the small intestine and ileo-cecal region are difficult regions in which to perform endoscopic biopsy for tissue diagnosis. CR and PR were achieved in 82% and 4% patients, respectively. The estimated 5-year OS and PFS rates were 86% and 54%, respectively. Regardless of stage, I-MZL was controlled relatively well with combined treatment.