Abstract

BackgroundSplenic marginal zone lymphoma (SMZL) is a rare B-cell malignancy, with no standard treatment other than splenectomy. The aim of this retrospective single-center study was to evaluate the clinical characteristics and prognosis of SMZL. MethodsBetween 2008 and 2015, we analyzed a total of 52 patients being considered as appropriate diagnostic criteria for SMZL in our hematology clinic. There were 36 (69%) female and 16 (31%) male patients. Median age was 58.8 years (range, 37-88 years). The median time of follow-up was 20.3 months (range, 3-97.7 months). ResultsWe retrospectively assessed 52 patients from single center, who received splenectomy, either alone or with chemotherapy. The presence of splenomegaly (95.2%) was recorded as the most frequent symptom at diagnosis. Of the patients, 44 (84.6%) had bone marrow involvement and 11 (21.2%) had lymph nodes involvement. Tumor involvement of peripheral blood defined as the presence of absolute lymphocytosis or 5% of tumor lymphocytes in peripheral blood was detected in 10 patients (19.2%). Because of the high frequency of bone marrow involvement, most patients in the series were Ann Arbor stage IV. Data concerning the presence of hypogammaglobulinemia was obtained from only 9 patients (42.9%). Obviously, 20 patients underwent splenectomy. In 7 patients (35%) chemotherapy was received apart from splenectomy.The number of patients reaching clinical complete remission after splenectomy with/out therapy was 18 (90%) and partial remission 3 (15%). The probability of 5 year-overall survival was 87.5%±11.7%. When we analyzed the patients with splenectomy alone the probability of overall survival was 83.3%±15.2%. ConclusionsIn conclusion, although the options of new treatment modality in SMZL have been debated today, splenectomy seems to be safe and effective in controlling long-term disease. Legal entity responsible for the studyThe authors. FundingHas not received any funding. DisclosureAll authors have declared no conflicts of interest.

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