Splenic Lymphoma with Villous Lymphocytes: Report of Three Cases

Abstract

Splenic lymphoma with villous lymphocytes (SLVL) is a recently recognized entity among chronic B lymphoproliferative disorders. It has a distinct clinical, morphological and immunophenotypic pattern and was previously described under a variety of designations. SLVL can be misdiagnosed as chronic lymphocytic leukemia (CLL), prolymphocytic leukemia (PLL), or hairy cell leukemia (HCL). Characteristically, these cells are CD5-, CD10-, CD23-, CD25-, HC2-, FMC7+, CD19+, CD22+ and strongly positive surface Ig (SmIg). Patients with SLVL are elderly (usually males) with splenomegaly and lymphocytosis (<100x 10 9 /L), with many villous lymphocytes, IgM monoclonal gammopathy in some, and usually have a benign course. We describe three cases of SLVL from the Eastern Province of Saudi Arabia. To our knowledge, this is the first well-documented report of SLVL from Saudi Arabia. Case Reports Case 1A 75-year-old Saudi male was first admitted to our hospital eight years ago. At that time, he had ischemic heart disease with inferior wall myocardial infarction and NIDDM. He had no splenomegaly and his CBC was normal. The patient was also known to have obstructive uropathy with hydronephrosis due to ureteric calculi, and had had an operation for this two years before that time. Four years ago, splenomegaly and lymphocytosis were noticed (Table 1), for which a diagnosis of CLL was made. Approximately three years ago, one of the authors (MIQ) noticed some villous lymphocytes in his blood film, and the patient was recalled for examination and evaluation. Examination showed a huge splenomegaly crossing the umbilicus. No lymphadenopathy was present. Ultrasonography showed the spleen was 20 cm long, the splenic vein was 1.4 cm, and there was bilateral hydronephrosis. Hemogram findings are shown in Table 1. The blood film showed varied forms of lymphoid cells. Some were large with abundant paleblue cytoplasm. The nuclei had clumped chromatin and prominent nucleolus in some. Some lymphocytes were binucleated and an occasional one was plasmacytoid. Several cells had fuzzy cytoplasm. Lobulated nucleus was seen in a few cells ( Figure 1A). There was another population of lymphoid cells which were comparatively small to medium in size but had short villi. These villi were either at one side (polar) (Figure 1B), or were located throughout the cell border. The percentage of such cells varied in different slides and on different occasions, but were between 10% and 30%. Some of the lymphoid cells were normal-looking small lymphocytes. A few smudge cells were also seen. The results of immunophenotype of blood lymphocytes using Becton Dickinson FAC SCAN are shown in Table 2. Serum protein electrophoresis showed no monoclonal gammopathy. Serum IgG/M/A were normal. Serum creatinine was slightly raised (1.99 mg/dL) and uric acid was 8.6 mg/dL. Bone marrow findings are shown in Table 3. The patient has shown a progression of the disease over three years of follow-up, and splenectomy is being considered.