First-line treatment with bendamustine and rituximab, in patients with intermediate-/high-risk splenic marginal zone lymphomas.

Splenic marginal zone lymphomas (SMZLs) are rare indolent B cell neoplasms that affect the spleen, bone marrow, and blood. Although they have an indolent course in the majority of patients, who have a median survival of 8-10 years, ∼ 30% may experience a worse outcome. The prognostic criteria of progression are lymph node and extra-nodal involvement, high lymphocyte counts, anaemia, and thrombocytopenia. The treatment of SMZLs include a "wait and watch strategy", splenectomy, and alkylating agents ± rituximab. We here describe data relating to 70 patients with intermediate-/high-risk SMZLs, who received rituximab/bendamustine as first-line treatment for a median of 60 days (range 1-75) after diagnosis. Sixty patients (86%) achieved a complete response (CR), and seven (10%) a partial response (PR). Three patients (4.3%) experienced disease progression (PD). The median duration of remission was 18 months. Side effects were generally mild. Our findings suggest that rituximab/bendamustine is a feasible treatment option in patients with intermediate-/high-risk SMZLs.