Follicular lymphoma presenting with marked splenomegaly: report of three cases.

Marked splenomegaly as the main presenting sign in follicular lymphoma (FL) is rare. The clinical and morphologic findings of 3 FL patients with massive splenomegaly and slight or no lymphadenopathy are presented. All cases had massive splenomegaly, and 2 had minimal peripheral lymphadenopathy with bone marrow infiltration, which is the major involved site besides the spleen. Histologically, various sizes of micronodules, composed of medium-sized centrocytes, were present in the white pulp in 2 cases in whom splenectomy was performed. The other case was complicated by nephrotic syndrome and showed aggregates of packed small lymphocytes in the spleen and renal parenchyma. Tumor cells were positive for CD10, CD20, bcl-2, and bcl-6. Since these cases are clinically similar to splenic marginal zone lymphoma, recognition of this disease and selection of the appropriate therapy are needed.