Treatment of splenic marginal zone lymphoma (SMZL) is not standardized due to the lack of prospective randomized trials. 1-13 After our initial 2007 paper, we now present updated data on the activity of rituximab as first-line treatment in a much larger series of SMZL patients (n 5 108) with a longer follow-up. 7 Diagnosis was established according to the World Health Organization and the Splenic Lymphoma Study Group (SLSG) consensus criteria. 14, 15 The study was approved by the appropriate institutional review boards. The simplified prognostic score as proposed by the SLSG was calculated at the time of diagnosis and before treatment initiation. 16-18 Criteria for rituximab initiation were bulky/symptomatic splenomegaly, cytopenias, B symptoms, and autoimmune manifestations. Treatment included an induction phase consisting of 6 weekly rituximab infusions (375 mg/m2). Responding patients either received rituximab maintenance every 2 months for 1 to 2 years or were followed up. Maintenance therapy was decided upon the practice of the treating physician within the 3 participating centers. Response assessment was scheduled at 2 months after the end of induction and was based on the SLSG consensus criteria. 15 In addition, the term unconfirmed complete response (CRu) was used to describe a complete response (CR) but without bone marrow reevaluation.

Freedom from progression (FFP) was defined as the time between rituximab initiation and disease progression. Deaths in remission due to unrelated causes were censored. Overall survival (OS) was defined as the time between rituximab initiation and death of any cause. Lymphoma-specific survival (LSS) was defined as the time between rituximab initiation and diseaserelated death. Maintenance vs no maintenance comparison was restricted to rituximab induction responders.