Rituximab with cyclophosphamide, vincristine, non-pegylated liposomal doxorubicin and prednisone as first-line treatment for splenic marginal zone lymphoma: a Fondazione Italiana Linfomi phase II study

Rituximab provides high response rates and effective disease palliation in patients with splenic marginal zone lymphoma (SMZL). We conducted a phase II trial in SMZL patients that were either untreated or were splenectomised, but had shown disease progression within 1 year after splenectomy. Treatment consisted of 6 courses of rituximab with cyclophosphamide, vincristine, non-pegilated liposomal doxorubicin and prednisone (R-COMP). Fifty-one patients were eligible for the analysis. The overall response rate was 84%. The 6-years progression free survival and overall survival was 54% and 72%, respectively. Toxicity was substantial (grade ≥3 neutropenia: 26%; grade ≥3 infections: 8%). Of the 15 deaths, 2 occurred on treatment (one sepsis and one pneumonia). Six deaths were due to lymphoma progression, 4 to secondary neoplasia, 1 to sepsis, 1 to pneumonia and one to splenectomy complications. R-COMP should be restricted to patients with bulky disease associated with symptoms or to patients with possible histological transformation