B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics

Seven patients with peripheral B-cell lymphoma associated with hemophagocyt ic syndrome are reported. In all cases, the histologic subtype was diffuse large B-cell lymphoma. Hemophagocytic features were noted in the bone marro w with lymphomatous infiltration. Hemophagocytic syndrome occurred with pre sentation of the lymphoma and was characterized by high fever, cytopenias, and elevated levels of lactate dehydrogenase, ferritin, C-reactive protein, and cytokines [interferon gamma, macrophage colony-stimulating factor, sol uble interleukin (sIL)-2R, and IL-6] without evidence of infection. The phe notypes of lymphomas were suspected CD19(+), CD20(+), S-Ig(+), CD10(-), and coexpression of CD5 in some cases. Flow cytometric analysis showed a low C D4/CD8 ratio in peripheral blood and bone marrow. We suggest that the patho genesis of hemophagocytic syndrome is hypercytokinemia induced by a prolife ration of reactive CD8(+) T cells. Previous reports of B-cell lymphoma with hemophagocytic syndrome demonstrated similar clinical manifestations and p oor prognoses. The invasion patterns of these diffuse large B-cell lymphoma s with hemophagocytosis may be classified into three groups: microscopic ly mph-node involvement type, gross lymph-node involvement type, and splenic l ymphoma type. Although hemophagocytic syndromes have been reported to be as sociated with T-cell lymphomas, our results indicate an association with di ffuse large B-cell lymphoma.