Current and emerging treatment approaches for splenic marginal zone lymphoma

ABSTRACT

Introduction: Splenic marginal zone lymphoma (SMZL) represents a distinct entity within the spectrum of marginal zone lymphomas (MZL). Due to its rarity, treatment is not standardized.

Areas covered: Currently two therapeutic options have been associated with the best outcome: splenectomy and rituximab. Splenectomy is associated with high response rates (~90%) with a median PFS>5 years. However, responses are not complete and it is associated with increased surgical risk as well as increased risk of septic episodes. Rituximab has shown significant efficacy with minimal toxicity in SMZL. The ORR is > 90%, with half of these responses being complete with a long duration of response. For the small proportion of SMZL with more aggressive clinical behavior, not responding or relapsing shortly after rituximab, novel agents are required. Several new drugs have shown significant activity in other low grade lymphomas. However, there is currently no trial testing the role of these agents specifically in SMZL.

Expert opinion: Rituximab monotherapy currently represents the best choice for first line treatment for SMZL. Randomized studies are required in order to improve the outcome, especially for the small proportion of cases with the more aggressive clinical behavior.

KEYWORDS:

• Splenic Marginal Zone Lymphoma
• treatment
• rituximab

Article highlights

• SMZL is a rare chronic lymphoproliferative disorder.

• Treatment is not standardized.

• Splenectomy and rituximab have been considered as the most effective first-line treatment strategies. Chemoimmunotherapy is more frequently used in relapsed/refractory disease.

• Novel agents have shown significant activity in many indolent B-cell lymphomas and can probably be effective in SMZL as well, especially in rituximab and chemotherapy refractory cases.

This box summarizes key points contained in the article.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Additional information

Funding

This paper was not funded.