Blood Res 2022; 57(S1):
Published online April 30, 2022
https://doi.org/10.5045/br.2022.2022054
© The Korean Society of Hematology
Correspondence to : Seong Hyun Jeong, M.D.
Department of Hematology-Oncology, Ajou University School of Medicine, 164 World Cup-ro, Yeongtong-gu, Suwon 16499, Korea
E-mail: seonghyunmd@naver.com
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.
Treatment of indolent lymphoma has improved significantly in recent decades since the advent of rituximab (anti-CD20 monoclonal antibody). Although, some patients with limited disease can be cured with radiation therapy alone, most patients experience disease progression and recurrence during follow-up despite early initiation of treatment. Thus, watch-and-wait is still regarded the standard for asymptomatic patients. Patients with indolent lymphoma have a significant heterogeneity in terms of tumor burden, symptoms (according to anatomical sites) and the need for instant therapy. Therefore, the initiation of treatment and treatment option should be decided with a clear goal in each patient according to the need for therapy and clinical benefits with the chosen treatment. In this review, we cover the current treatment of follicular lymphoma and marginal zone lymphoma.
Keywords Indolent lymphoma, Non-Hodgkin’s lymphoma, Follicular lymphoma, Marginal zone lymphoma
Blood Res 2022; 57(S1): S120-S129
Published online April 30, 2022 https://doi.org/10.5045/br.2022.2022054
Copyright © The Korean Society of Hematology.
Seong Hyun Jeong
Department of Hematology-Oncology, Ajou University School of Medicine, Suwon, Korea
Correspondence to:Seong Hyun Jeong, M.D.
Department of Hematology-Oncology, Ajou University School of Medicine, 164 World Cup-ro, Yeongtong-gu, Suwon 16499, Korea
E-mail: seonghyunmd@naver.com
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.
Treatment of indolent lymphoma has improved significantly in recent decades since the advent of rituximab (anti-CD20 monoclonal antibody). Although, some patients with limited disease can be cured with radiation therapy alone, most patients experience disease progression and recurrence during follow-up despite early initiation of treatment. Thus, watch-and-wait is still regarded the standard for asymptomatic patients. Patients with indolent lymphoma have a significant heterogeneity in terms of tumor burden, symptoms (according to anatomical sites) and the need for instant therapy. Therefore, the initiation of treatment and treatment option should be decided with a clear goal in each patient according to the need for therapy and clinical benefits with the chosen treatment. In this review, we cover the current treatment of follicular lymphoma and marginal zone lymphoma.
Keywords: Indolent lymphoma, Non-Hodgkin’s lymphoma, Follicular lymphoma, Marginal zone lymphoma
Table 1 . Indications for treatment in low grade lymphoma..
Indication | Detail |
---|---|
High tumor burden [10] | Any site >7 cm |
Three or more sites >3 cm | |
Splenomegaly (>16 cm) | |
Pleural or peritoneal effusion | |
Circulating tumor cells >5,000/mL | |
Cytopenia secondary to lymphoma | |
- Absolute neutrophil count <1,000/mL | |
- Platelet count <100,000/mL | |
Disease-related symptoms | Fever |
Night sweats | |
Weight loss | |
Compression | |
Other lymphoma-related symptoms | |
Steady progression | Over at least 6 months |
Table 2 . Treatment of follicular lymphoma..
Disease status | Treatment | Comment |
---|---|---|
Localized disease | RT | - Potentially curative (ISRT 24–30Gy) |
- The addition of systemic therapy to RT improves PFS but not OS | ||
Rituximab | - Radiotherapy ineligible patients | |
CIT | - Non-contiguous, bulky disease | |
Watch and wait | - Stable, asymptomatic patients | |
Advanced disease | Watch and wait | - Without treatment indications (Table 1) |
CIT± antibody maintenance | - Rituximab or obinutuzumab+(CHOP, CVP, Bebdamustine) | |
- Maintenance improves PFS but not OS | ||
Rituximab Lenalidomide+rituximab | - For low tumor burden | |
- As effective as chemoimmunotherapy | ||
Relapsed disease | Watch and wait | - Stable, asymptomatic patients |
Palliative RT | - 2×2Gy | |
CIT± antibody maintenance | - Long previous remission with CIT | |
- Non-resistant regimen | ||
Rituximab | - For low tumor burden | |
Lenalidomide+rituximab | - POD≤24 months after CIT | |
PI3K inhibitors | - Double refractory disease | |
EZH2 inhibitor (tazemetostat) | - EZH2 mutation-positive disease | |
Radioimmunotherapy | - Not widely used | |
Auto/allo-HSCT | - In selected patients | |
CAR-T cell therapy | - After ≥2 lines of systemic therapy [63] |
Abbreviations: CAR-T, chimeric antigen receptor T-cell; CIT, chemoimmunotherapy; CR, complete response; EZH2, enhancer of zeste homolog 2; HSCT, hematopoietic stem cell transplantation; ISRT, involved site RT; ORR, overall response rate; OS, overall survival; PFS, progression-free survival; PI3K, phosphatidylinositol 3-kinase; POD, progression of disease; RT, radiotherapy..
Table 3 . Treatment of marginal zone lymphoma..
Disease | Treatment | Comment |
---|---|---|
Gastric ENMZL | H. pylori eradication | - PPI+clarithromycin+(amoxicillin or metronidazole) |
RT | - H.pylori(-), or eradication failure | |
Rituximab | - For radiotherapy ineligible patients | |
Gastrectomy | - Major gastric bleeding | |
Non-gastric ENMZL | Watch and wait | - Stable asymptomatic disease |
Targeting infectious agents | - HCV treatment for HCV(+) disease | |
- Doxycycline for ocular adnexal ENMZL | ||
RT | - Definitive or palliative | |
Rituximab | - Higher response in CTx-naïve patients | |
CIT | - R-chlorambucil, R-bendamustine | |
Lenalidomide+Rituximab | - To avoid chemotherapy | |
Surgery | - Mostly for diagnosis (thyroid, breast, intestine, etc.) | |
Splenic MZL | Watch and wait | - Stable asymptomatic disease |
HCV eradication | - For HCV(+) disease | |
Rituximab | - Offer the most risk/benefit ratio [106] | |
Splenectomy | - After rituximab failure | |
CIT | - For symptomatic disseminated disease after rituximab or splenectomy failure | |
Nodal MZL | Treated as guidelines for FL | - Studies enrolled solely MZL are rare |
Abbreviations: CIT, chemoimmunotherapy; CTx, chemotherapy; ENMZL, extranodal marginal zone lymphoma; HCV, hepatitis C virus; PPI, proton-pump inhibitor; RT, radiotherapy..
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