A "floral" variant of nodal marginal zone lymphoma

We describe 6 cases of a specific variant of nodal marginal zone lymphoma with "floral" lymph follicles in patients ranging in age from 18 to 66 years. All 6 patients had lymphadenopathy, either local (n = 5) or systemic (n = 1), and good performance status (0), and none had fever, weight loss, or night sweating. They all underwent excisional biopsy. Histologically, all lesions had a distinctive morphology, with proliferation of medium-sized atypical lymphoid cells in the marginal zone, hyperplastic lymph follicles with enlarged germinal centers, and a thickened mantle zone. In places, folliculolysis was observed. On immunohistochemical staining, the atypical lymphoid cells showed a B-cell phenotype (CD20 +), IgM positivity in 2 of 5 cases, and negativity for CD5, CD10, CD23, CD43, bcl-6, and IgD. Polymerase chain reaction examination for immunoglobulin heavy chain in 5 cases showed monoclonality in all. Five patients did not receive adjuvant chemotherapy and had no recurrences. The patient with systemic lymphadenopathy received chemotherapy and had a complete response without relapse. This variant should be differentiated from the usual nodal marginal zone lymphoma because of its specific clinical and pathological features.