Primary splenic lymphoma: report of 10 cases using the REAL classification

Primary splenic lymphoma (PSL) is rare with a reported incidence of less than 1%. Diffuse large cell pathology has been reported in 22-33% of the cases and is felt to have a poor outcome. We report our experience in patients with PSL seen at Mount Sinai Medical Center during the years 1994-1999. Our objective was to evaluate staging (using the Ahmann and Kehoe criteria), prognosis using the International Prognostic Index (IPI), and pathology using the Revised European-American Lymphoma Classification (REAL) classification. Ten patients were identified. Eight of the 10 patients had diffuse large cell lymphoma (DLCL). Using the IPI, four patients were categorized as low risk, three as low/intermediate risk, and three as high risk. The only two deaths occurred in the high-risk group. Lymph node involvement beyond the splenic hilum seen by imaging studies represents an advanced non-Hodgkin's lymphoma and should be included no longer in the staging of PSL. Nine of the 10 underwent a splenectomy. Eight of the nine patients received chemotherapy following splenectomy. Seven of the nine patients remained in remission from 1 to 19 years. Splenectomy followed by combination chemotherapy, results in excellent long-term survival in PSL.