FROM BEST PRACTICE & RESEARCH CLINICAL HAEMATOLOGY

For patients with splenic marginal zone lymphoma (MZL) requiring treatment, rituximab may be a superior choice, compared with splenectomy, according to authors of a recent review article.

Although treatment with splenectomy and rituximab both are associated with high rates of 10-year survival, splenectomy also is associated with acute surgical complications and late toxicities, mainly from infections, Christina Kalpadakis, MD, of the department of hematology at Heraklion University Hospital, University of Crete, Greece, reported in Best Practice & Research Clinical Haematology .

The review article aims to answer the question of whether rituximab monotherapy should replace splenectomy as the treatment of choice, according to the authors, who said treatment for this rare, low-grade B-cell lymphoma has not been standardized due to a lack of large, randomized trials.

Asymptomatic splenic MZL without significant cytopenias can be managed with a watch-and-wait approach, but when treatment is warranted, the choice is often between splenectomy, rituximab monotherapy, or chemoimmunotherapy.

“Based on the existing retrospective series of patients, rituximab monotherapy appears to be the best choice since it combines high efficacy with the lowest toxicity,” Dr. Kalpadakis and her colleagues wrote.

Until the early 2000s, splenectomy was the standard treatment modality for splenic MZL, offering quick amelioration of symptoms related to splenomegaly in more than 90% of patients, as well as improvements in hypersplenism-related cytopenias, the researchers reported. Among 100 patients with splenic MZL, the median progression-free survival with splenectomy was 8.25 years, and median 10-year overall survival was 67%.

However, responses to splenectomy are not complete because some patients are not good candidates for splenectomy, including those with lymphadenopathy or heavy bone marrow infiltration, according to the authors. Moreover, since it is a major surgical procedure that can be associated with complications and infections, splenectomy is not appropriate for elderly patients or patients with comorbidities with a high surgical risk, they wrote.

“Furthermore, splenectomy cannot eradicate bone marrow disease and has no impact on other extrasplenic disease localization such as lymphadenopathy,” they added.

Rituximab monotherapy, by contrast, has “minimal toxicity” with high efficacy, including complete response rates of around 50% and a 10-year overall survival of 85% reported in a series of 104 patients.

Maintenance with rituximab may further improve the quality of responses, although data are limited. One study, conducted by the review article authors, showed that rituximab maintenance increased the complete response rate from 42% after induction treatment to 71% after maintenance, along with a significant improvement in response duration, though no differences in overall survival have been observed thus far.

Rituximab plus chemotherapy has been evaluated, but with “significant toxicity without improvement in the outcome” versus rituximab monotherapy, the researchers wrote. Splenectomy is “effective” but should be reserved for patients refractory to rituximab, Dr. Kalpadakis and her colleagues wrote.

The researchers reported having no relevant financial disclosures.

hematologynews@frontlinemedcom.com

SOURCE: Kalpadakis C et al. Best Pract Res Clin Haematol. Mar-Jun 2017. doi:10.1016/j.beha.2017.10.011 .

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