[Clinical characteristics of splenic marginal zone lymphoma with abnormal complete blood count]

The aim of this study was to investigate the clinical and laboratorial characteristics of splenic marginal zone lymphoma (SMZL) with an abnormal complete blood count (CBC). Data of 19 newly diagnosed SMZL patients with abnormal CBC were analyzed retrospectively. Seven patients were diagnosed by using splenic histology, 12 patients who did not undergo splenectomy were diagnosed on the basis of typical clinical presentation and cytologic, immunophenotypic and histologic characteristics of peripheral blood and bone marrow, according to SBLG guidelines. The results showed that leukocytosis (≥ 10.0×10(9)/L) was seen in 5 cases (26.3%); leukocytopenia (< 4.0×10(9)/L) was found in 6 cases (31.6%), hemoglobin concentration less than 120 g/L was found in 14 cases (73.7%) and thrombocytopenia was found in 11 (57.9%) patients. Fourteen (73.7%) patients had cytopenia in one or more lineage. As a specific morphologic character, villous lymphocytes were found in 10 (52.6%) patients. Similar immunophenotype was determined by histology in both bone marrow and spleen. Various histological infiltration patterns including intrasinusoidal pattern were found in bone marrow. Nine out of 16 (56.3%) patients displayed an increase of serum monoclonal immunoglobin. Autoimmune phenomena was found in 12 out of 15 (80.0%) patients. Splenectomy, as the only treatment could not achieve a ≥ 50% improvement of CBC in 4 patients, and then was judged as no response. Splenectomy followed by chemotherapy achieved partial response (PR) in 1 patient. Overall response rate of the therapeutic strategies with Rituximab was 100.0% (11/11). Furthermore, complete response was achieved in 9 out of 11 (81.8%) patients. It is concluded that SMZL with abnormal CBC has a higher incidence of cytopenia, bone marrow involvement and autoimmune phenomena. Therapeutic strategies consisting of Rituximab show a better efficacy.