After completing this course, the reader will be able to:1. Describe the effect of the addition of rituximab to standard CHOP chemotherapy on the outcome of patients with primary mediastinal large B-cell lymphoma.2. Explain potential changes in the use of radiotherapy and aggressive chemotherapy in the rituximab era.This article is available for continuing medical education credit at CME.TheOncologist.com. CME CME

Summary The prognostic value of baseline serum free light chain ratio (sFLCR) was investigated in 94 multiple myeloma (MM) patients. sFLCR was calculated as κ/λ or λ/κ, depending on the patients’ dominating monoclonal light chain. Median baseline sFLCR was 3·57 in κ‐MM patients, 45·09 in λ‐MM. ‘High’ sFLCR (≥ the observed median value for κ‐ and λ‐MM respectively) correlated with elevated serum creatinine and lactate dehydrogenase, extensive marrow infiltration and light chain type MM. The 5‐year disease‐specific survival was 82% and 30% in patients with sFLCR lower than and equal or greater than the median, respectively (P = 0·0001). sFLCR was an independent prognostic factor.

Key Points• Clonal B-cell lymphocytosis of potential marginal-zone origin (CBL-MZ) rarely progresses to a well-recognized lymphoma.• CBL-MZ does not require treatment in the absence of progressive disease. The clonal B-cell count, degree of marrow infiltration, immunophenotypic, or immunogenetic findings at diagnosis did not distinguish between the 2 groups. However, deletions of chromosome 7q were confined to group A and complex karyotypes were more frequent in group B. Although CBL-MZ may antedate SMZL/SLLU, most cases remain stable over time. These cases, not readily classifiable within the World Heath Organization classification, raise the possibility that CBL-MZ should be considered as a new provisional entity within the spectrum of clonal MZ disorders. (Blood. 2014;123(8):1199-1206

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...