Primary isolated bone marrow disease as a presenting feature of diffuse large B-cell lymphoma is very rare. We describe the first Korean case of isolated bone marrow diffuse large B-cell lymphoma with hemolytic anemia as the first manifestation. A32-year-old man was admitted to our hospital presenting with fever and hematuria. He had severe anemia and high lactate dehydrogenase activity. His peripheral blood smear and laboratory findings were suggestive of intravascular hemolytic anemia. The bone marrow biopsy revealed involvement with diffuse large B-cell lymphoma. A computed tomographic scan showed splenomegaly, but no lymphadenopathy. Our case shared some clinical features with the Asian variant of intravascular B-cell lymphoma, but there was infrequent involvement of the sinusoids of lymphoma cells and no hemophagocytosis. Our patient was treated with R-CHOP regimen for six cycles and he is in remission after autologous peripheral blood stem cell transplantation.

Keywords: Diffuse large B-cell lymphoma, Bone marrow, Hemolytic anemia