Treatment of Splenic Marginal Zone Lymphoma With Rituximab Monotherapy: Progress Report and Comparison With Splenectomy

Abstract

Background. Treatment of splenic marginal zone lymphoma (SMZL) patients is not standardized. Recent data suggest that rituximab is highly effective and could be considered as initial therapy. Aim. To assess the efficacy of rituximab monotherapy in a large series of patients with SMZL and compare these results with splenectomy results. Methods. The studied population included 85 patients. Fiftyeight received rituximab at a dose of 375 mg/m 2 per week for 6 weeks as induction followed by maintenance at the same dose every 2 months for 1-2 years, whereas 27 patients were treated using splenectomy only. Results. The overall response rate to rituximab 2 months after the end of induction was 95% (complete response [CR], 45%; unconfirmed CR, 26%; partial response, 24%). The median times to hematologic and clinical response were 2 weeks and 3 weeks, respectively. Forty-three of 55 patients already completed the maintenance phase: 28 sustained their initial response, 14 improved their response, and one progressed. Eighty-five percent of splenectomized patients responded, and two were treated with rituximab as consolidation after splenectomy and achieved a CR. The 5-year overall and progression-free survival (PFS) rates for rituximab-treated and splenectomized patients were 92% and 77% (p ϭ .09) and 73% and 58% (p ϭ .06), respectively. Furthermore, maintenance therapy with rituximab resulted in a longer duration of response (at 5 years, PFS was 84% for patients receiving maintenance and 36% for patients without maintenance, p Ͻ.0001). Conclusions. Rituximab is a very effective and well-tolerated therapy and may be substituted for splenectomy as the firstline treatment of choice for patients with SMZL. TheOncologist 2013;18:190 -197 Implications for Practice: Treatment in splenic marginal zone lymphoma (SMZL) is not standardized. Splenectomy has been considered as the treatment of choice in symptomatic patients. However, this can only result in a partial response. Furthermore, splenectomy is a major surgical procedure with significant morbidity, especially in elderly patients. Recent data, in a relatively small series of patients, suggests that the monoclonal antibody anti-CD20, rituximab, is highly effective with minimal toxicity. In this study, we compare these two treatment modalities in a large number of patients with long follow up. Our data shows that rituximab is associated with better quality of response as almost half of the patients achieved CR in contrast to splenectomized patients, along with better five-year PFS and OS. Furthermore, we stress the importance of maintenance therapy, since it can clearly improve the duration of remission. Based on these findings, we suggest that rituximab should be the treatment of choice in SMZL. INTRODUCTIONSplenic marginal zone lymphoma (SMZL) is a rare form of indolent B-cell lymphoma, accounting for Ͻ2% of all lymphoid malignancies [1,2]. It mainly affects elderly or middle-aged patients, with a median age of ϳ65 years. SMZL is characterized by splenomegaly withou...

Splenic marginal zone lymphoma: from genetics to management

“…15,66 No randomized trials have been conducted in SMZL and, as consequence, there is no consensus on how to treat newly diagnosed and relapsed patients. The therapeutic options for SMZL have a wide range and include splenectomy, 1,7,59,61,[68][69][70][71][72][73] chemotherapy, [74][75][76][77][78][79][80][81] and rituximab alone 70,82,83 or rituximab with chemotherapy. 70,[84][85][86][87] In addition, antiviral treatment should be considered in patients with SMZL and concurrent chronic infection with HCV-related hepatitis who do not need immediate conventional treatment against the lymphoma.…”

Splenic marginal zone lymphoma with autoimmune hemolytic anemia

“…Rituximab monotherapy is equally effective with lesser side effects [4,23] and has dual action on both AIHA and lymphoma remission with minimal apparent toxicity [24]. However, our patient was reactive to Hepatitis B core antibody and rituximab therapy risks revitalization of hepatitis B virus.…”

“…immunopheotypic features (positive markers: CD19, CD 29a, HLA-DR, Bright CD20, FMC7, sIgM, sIgD and sIgM, negative markers: CD5, CD10, CD25, CD34, CD38, CD43, CD103, sIgG lambda) and finally presence of AIHA [3][4][5][6][7][8][9][10][11][12].…”

“…There is no specific immunophenotypic marker for SMZL, with lymphoma cells expressing pan-B-cell antigens, usually CD20, CD45RA, CD45RB, CD79a, PAX5/BSAP, IgM, and surface IgM and/or Ig D, but negative for CD5, CD10, and CD23, CD43, bc16 and cyclin D1. B symptoms (weight loss, night sweats, fever) are rare [3][4][5].…”

Synchronous Rectal Adenocarcinoma and Splenic Marginal Zone Lymphoma

“…However, symptomatic patients are currently treated primarily with rituximab alone or in combination with chemotherapy, with or without splenectomy, depending on symptom severity 16 . Post-splenectomy progression-free survival rates at 4-5 years are approximately 58%-80%, with a 5-year overall survival of 72%-88% [16][17][18][19][20][21][22] . Single-agent rituximab therapy was recently found to result in an overall response rate of 88%-100%, with a progression-free survival rate at 4-5 years of 60%-73%, and a 5-year overall survival rate of 81%-92% 16,17,21,23,24 .…”