[Splenic lymphoma with circulating villous lymphocytes].

Splenic lymphoma with circulating villous lymphocytes (SLVL) is a special kind of lymphoproliferative disease characterized by specific clinical, haematological, histomorphological and immunophenotypic features that make it different from hairy cell leukaemia, prolymphocytic leukaemia and non-Hodgkin lymphoma. Four patients suffering from SLVL, 3 men and 1 woman, 62 years in average (range 55-67 years), are presented. All patients had anaemia and splenomegaly. One patient had huge pseudocyst of the spleen, two had mild lymph-adenopathy, while 3 patients had thrombocytopenia. The number of WBC ranged from 13-29 x 10(9)/l with lymphocytes ranging from 62-80%. Monoclonal IgM paraprotein was found in 3 patients. Immunophenotyping showed cells with features of mature B lymphocytes. Splenectomy was carried out in three patients. They all recovered and stayed symptomfree. One patient refused surgery. In our opinion SLVL is not a rare disease as it was thought; it should especially be taken into diagnostic consideration in elder patients, particularly in males with splenomegaly and lymphocytosis.