How do we sequence therapy for marginal zone lymphomas?

Abstract

Marginal zone lymphomas are indolent diseases. Overall survival rates are very good, but patients tend to relapse and may do so several times. The concept of treatment sequencing is therefore important and necessary to preserve adequate organ function and to avoid excessive toxicity, with the final goal of achieving long survival times. Systemic treatments and chemotherapy are considered to be an option in multiply relapsing disease, in cases that are in an advanced stage at presentation or relapse, and in cases where initial local treatments lack efficacy. Targeted agents and new drugs can provide chemotherapy-free alternatives in heavily pretreated patients.

Prognostic significance of serum immunoglobulin paraprotein in mucosa‐associated lymphoid tissue (MALT) lymphoma

“…Exposure to such effective therapies needs a powerful prognostic tool for sequencing of available therapies to avoid excessive toxicity. 21 In our study, Ig paraproteinaemia together with MALT-IPI was shown to improve our ability to identify high-risk populations, which is critically important in therapeutic management.…”

Clinical experiences with venetoclax and other pro-apoptotic agents in lymphoid malignancies: lessons from monotherapy and chemotherapy combination

“…The median PFS and DOR were 12 and 10 months, respectively [ 86 ]. As seen with other therapeutic advances, dedicated analyses of this indolent subtype are made difficult by their relatively rarity and insights are largely extrapolated from broader cohorts of B cell malignancies [ 104 ].…”

Current Status of the Spectrum and Therapeutics of Helicobacter pylori-Negative Mucosa-Associated Lymphoid Tissue Lymphoma

“…Systemic therapy is often prescribed in localized patients who cannot tolerate the adverse effects of radiotherapy, or in those with advanced or disseminated MALT lymphoma [ 10 , 20 , 21 , 23 , 105 ]. Previously, several phase II studies evaluating new purine analogs of chemotherapy agents, such as fludarabine and 2CdA, provided approximately 50% ORRs, but these drugs caused myelosuppression [ 10 , 20 , 21 , 23 , 105 , 106 ].…”

“…Systemic therapy is often prescribed in localized patients who cannot tolerate the adverse effects of radiotherapy, or in those with advanced or disseminated MALT lymphoma [ 10 , 20 , 21 , 23 , 105 ]. Previously, several phase II studies evaluating new purine analogs of chemotherapy agents, such as fludarabine and 2CdA, provided approximately 50% ORRs, but these drugs caused myelosuppression [ 10 , 20 , 21 , 23 , 105 , 106 ]. In a randomized International Extranodal Lymphoma Study Group (IELSG)-19 trial comparing chlorambucil, rituximab, and combined chlorambucil and rituximab as first-line treatments for patients with MALT lymphoma, Zucca et al showed that the ORR for chlorambucil, rituximab, and combined chlorambucil and rituximab arms were 85.5%, 78.3%, and 94.7%, respectively [ 107 ].…”

“…However, for extragastric MALT lymphomas that are not associated with bacterial infection, the use of radiotherapy (RT) as first-line treatment for localized disease and administration of systemic therapy, including chemotherapy or rituximab for advanced and metastatic diseases, remains the standard therapy [ 10 , 20 , 21 ]. Considering that the biological behaviors of localized HP-negative gastric MALT lymphoma and extragastric MALT lymphoma are relatively indolent, alternative treatments with less toxic strategies for treating these diseases have been explored [ 22 , 23 , 24 , 25 ].…”