We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenström's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.
From the Departments of Pathology (J.-P.D.V.H., T.M., J.A., A.L.T., J.D.), Hematology (A.D., R.Z.), and Oncology (A.B.), Hôtel Dieu, AP-HP, Paris, France.
Address correspondence and reprint requests to Professor Jacques Diebold, MD, Laboratoire “Jacques-Delarue” d'Anatomie et de Cytologie Pathologiques, H|f.tel-Dieu, AP-HP, 1 Place du Parvis Notre Dame, F. 75181 Paris cedex 04, France; e-mail:firstname.lastname@example.org