SPLENIC LYMPHOMA WITH CIRCULATING VILLOUS LYMPHOCYTES - REPORT OF 7 CASES AND REVIEW OF THE LITERATURE

Splenic lymphoma with villous lymphocytes (SLVL) is a relatively new e ntity with only a few reports published. We report seven cases of SLVL with detailed clinicopathologic and comprehensive immunophenotypic st udies to further characterize this lymphoma, which is frequently confu sed with hairy cell leukemia and other low-grade B-cell lymphoid neopl asms. The diagnostic criteria we used include 1) prominent splenomegal y, 2) insignificant or no lymphadenopathy, 3) lymphocytosis without le ukopenia, 4) presence of circulating villous lymphocytes, 5) character istic cytologic and histologic features, and 6) specific phenotypic an d cytochemical findings. Our studies show that SLVL does not represent a pure entity but rather a morphologically heterogeneous group of low -grade lymphomas with various cytologic and histologic features. Altho ugh immunophenotyping is helpful in differential diagnosis, multiparam eter studies are necessary to confirm the diagnosis. In our series, on ly two patients died of SLVL, who probably developed transformation to a higher-grade lymphoma. (C) 1994 Wiley-Uss, Inc.