An autopsy case of splenic lymphoma with villous lymphocytes with rapidly decreased splenomegaly and extremely increased peripheral villous lymphocytes

A 81-year-old Japanese female was referred to our hospital with fever up, lumbago and marked splenomegaly (10cm below the left costal margin) without lymphadenopathy. Laboratory findings showed leukocytosis (60.5×109/l), mild thrombocytopenia (89×109/l) and M-proteinemia (total protein 8.1g/dl, IgG 5.469g/dl, IgG-λ positive). 92% of leukocytes were villous lymphocytes and the tartrate-resistant acid phosphate reaction was negative. The immunophenotypes of these cells were CD3-, CD5-, CD10-, CD11c+, CD19+, CD20+, CD22+, CD25-, FMC7+, MikB1+, sIgM+. The DNA analysis of the immunoglobulin gene of the peripheral blood showed rearrangement of JH gene. The first lumbar vertebra was fractured. We diagnosed splenic lymphoma with villous lymohocytes, started radiation to bone involvement and chemotherapy with VMCP regimen, but splenomegaly increased to the pelvis and leukocytosis persisted. Furthermore, serum electropheresis showed biclonal gammopathy (IgG-λ, IgM-λ), and the immunophenotypes of villous lymphocytes revealed IgG-κ and IgM-κ potitive.After two years she complained abdominal pain. Splenomegaly decreased rapidly and peripheral villous lymphocytes increased extremely to 150×109/l without blastic transformation. Abdominal computed tomography showed decreased splenomegaly with a few nodules of low density and slight ascites. Splenic infarction or rupture were not showed. Metabolic acidosis, hypoglycemia and paralytic ileus occured. She died next day.Autopsy showed systemic intravascular and severe extravascular infiltrations of the villous lymphocytes. The parenchyma of spleen was involved with severe lymphoid infiltration (μ+, γ+, α-, κ+, λ-) and white pulp was dissappeard. Bone marrow also showed the plasmacytoid cells (γ+ [>>μ+], λ+, κ-). Severe acute ischemic enterocolitis without thrombosis was seen in small intestine and colon. We suspect that rapidly increased villous lymphocytes came to peripheral circulatory insufficiency, but we couldn't know the relationship between decreased splenomegaly and rapidly increased villous lymphocytes.