MALT lymphoma is an indolent (low-grade) lymphoma and outcomes are generally favorable, particularly if disease is localized.[67]Cheah CY, Zucca E, Rossi D, et al. Marginal zone lymphoma: present status and future perspectives. Haematologica. 2022 Jan 1;107(1):35-43.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8719063
http://www.ncbi.nlm.nih.gov/pubmed/34985232?tool=bestpractice.com
Poor prognostic factors
These include poor performance status at time of diagnosis, the presence of t(11;18) translocation, and high-grade transformation. The t(11;18) translocation is associated with Helicobacter pylori-negative MALT tumors, poor response to H pylori eradication therapy, and a high incidence of presentation in advanced stage.
MALT International Prognostic Index (MALT-IPI)
MALT-IPI is a validated prognostic tool for gastric and nongastric MALT lymphoma based on the following poor prognostic factors:[68]Thieblemont C, Cascione L, Conconi A, et al. A MALT lymphoma prognostic index. Blood. 2017 Sep 21;130(12):1409-17.
https://www.sciencedirect.com/science/article/pii/S0006497120328470?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/28720586?tool=bestpractice.com
A revised version of MALT-IPI includes multiple mucosal sites as an additional poor prognostic factor.[69]Alderuccio JP, Reis IM, Habermann TM, et al. Revised MALT-IPI: A new predictive model that identifies high-risk patients with extranodal marginal zone lymphoma. Am J Hematol. 2022 Dec;97(12):1529-37.
https://pmc.ncbi.nlm.nih.gov/articles/PMC9847507
http://www.ncbi.nlm.nih.gov/pubmed/36057138?tool=bestpractice.com
Eradication therapy in localized H pylori-positive gastric MALT lymphoma
H pylori eradication therapy alone induces long-term remission in >60% of patients with localized H pylori-positive gastric MALT lymphoma.[70]Wündisch T, Thiede C, Morgner A, et al. Long-term follow-up of gastric MALT lymphoma after Helicobacter pylori eradication. J Clin Oncol. 2005 Nov 1;23(31):8018-24.
http://www.ncbi.nlm.nih.gov/pubmed/16204012?tool=bestpractice.com
[71]Fischbach W, Goebeler-Kolve ME, Dragosics B, et al. Long term outcome of patients with gastric marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT) following exclusive Helicobacter pylori eradication therapy: experience from a large prospective series. Gut. 2004 Jan;53(1):34-7.
https://pmc.ncbi.nlm.nih.gov/articles/PMC1773912
http://www.ncbi.nlm.nih.gov/pubmed/14684573?tool=bestpractice.com
Remission rate is significantly higher in:[72]Zullo A, Hassan C, Cristofari F,et al. Effects of Helicobacter pylori eradication on early stage gastric mucosa-associated lymphoid tissue lymphoma. Clin Gastroenterol Hepatol. 2010;8:105-110.
http://www.ncbi.nlm.nih.gov/pubmed/19631287?tool=bestpractice.com
Patients with stage I lymphoma (compared with stage II lymphoma [78.4% vs. 55.6%, respectively])
Asian people (compared with white people [84.1% vs. 73.8%, respectively])
Lymphoma localized to the distal stomach (compared with proximal stomach [91.8% vs. 75.7%, respectively])
The remission rate is higher in patients without the t(11;18) translocation compared with those with the translocation (78% vs. 22.2%, respectively).[72]Zullo A, Hassan C, Cristofari F,et al. Effects of Helicobacter pylori eradication on early stage gastric mucosa-associated lymphoid tissue lymphoma. Clin Gastroenterol Hepatol. 2010;8:105-110.
http://www.ncbi.nlm.nih.gov/pubmed/19631287?tool=bestpractice.com
The length of time to achieve remission is variable, but may be >1 year.[23]National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: B-cell lymphomas [internet publication].
https://www.nccn.org/guidelines/category_1
[73]Zucca E, Cavalli F. Are antibiotics the treatment of choice for gastric lymphoma? Curr Hematol Rep. 2004;3:11-16.
http://www.ncbi.nlm.nih.gov/pubmed/14695844?tool=bestpractice.com
Radiation therapy in H pylori-independent gastric MALT lymphoma
In patients with H pylori-independent gastric MALT lymphoma (all stages), complete pathologic response rate with radiation therapy is 95%, with 5-year and 10-year overall survival rates of 94% and 79%, respectively.[74]Yahalom J, Xu AJ, Noy A, et al. Involved-site radiotherapy for Helicobacter pylori-independent gastric MALT lymphoma: 26 years of experience with 178 patients. Blood Adv. 2021 Apr 13;5(7):1830-6.
https://pmc.ncbi.nlm.nih.gov/articles/PMC8045489
http://www.ncbi.nlm.nih.gov/pubmed/33787863?tool=bestpractice.com
Chemoimmunotherapy in advanced-stage MALT lymphoma
In patients with advanced-stage MALT lymphoma, complete response rate with chemoimmunotherapy (rituximab plus cyclophosphamide, vincristine, and prednisone [R-CVP]) is 60%, with estimated 3-year progression-free survival and overall survival rates of 59% and 95%, respectively.[75]Kang HJ, Kim WS, Kim SJ, et al. Phase II trial of rituximab plus CVP combination chemotherapy for advanced stage marginal zone lymphoma as a first-line therapy: Consortium for Improving Survival of Lymphoma (CISL) study. Ann Hematol. 2012 Apr;91(4):543-51.
http://www.ncbi.nlm.nih.gov/pubmed/21922208?tool=bestpractice.com