Purpose: The splenic lymphoma with circulating villous lymphocytes (SLCVL) is an infrequent disease included within the low grade non Hodgkin's lymphoma, B-cell type. The results of the study of four patients are reported.
Patients and methods: Clinical, cytological, immunophenotypic, ultrastructural, evolutive and therapeutic data have been revised in all the cases.
Results: Two males and 2 females of 76, 66, 68 and 62 years, respectively were diagnosed as having SLCVL. The initial symptoms were scarce, basically asthenia, and a big spleen without significant lymphadenopaty was the most relevant physical finding in each of them. In peripheral blood leukocyte count was normal with a slight lymphocytosis and a variable percentage of villous circulating lymphocytes. The immunophenotype of peripheral blood obtained by flow cytometry was according with a mature B-cell lymphocyte population, CD 5 and CD 25 negative. The cells were positive to acid phosphatase with a diffuse pattern of variable intensity; the reaction was inhibited by tartaric acid. All the patients had BM infiltration, studied with aspiration and biopsy. One case (M,66) had an IgM monoclonal gammopathy. The ultrastructural study, performed in 3 cases, showed thin and short villous prolongations. After splenectomy, a low degree lymphoma therapy has been employed in all the cases. The follow-up ranges between, 4 years and 4 months, all the patients being alive.
Conclusions: The SLCVL is a definitive entity regarding the clinical, morphologic and immunophenotype features. A long clinical evolution and a good prognosis after splenectomy are common.