RESUMO
Objective To study the clinical and pathologic features, diagnosis, treatment and prognosis of splenic marginal zone lymphoma (SMZL). Methods We reported the detailed clinical and pathologic presentation of a patient with SMZL, and monitored fellow-up to 16 years. Results Patients present with splenomegaly. SMZL usually involve bone marrow, peripheral blood and hilar splenic lympha nodes. Peripheral lymph nodes and nonhematolymphoid organs can be involved as well, but to a lesser extent.Microscopically, tumor cells are small to medium in size, with dispersed chromation. Tumor cells are slgM(+),CD+20, CD+79a,, bcl-2(+), CD-3, CD-5, CD-10, CD-23 and CyclinDl(-). Conclusion SMZL is a rare low-grade B-cell lymphoma with an indolent clinical course. Spleneetomy and chemotherapy are effective treatment but SMZL cannot be completely cured and may transform to large B-cell lymphoma at last.