Angiotropic B-cell lymphoma with hemophagocytic syndrome

A unique autopsy case of angiotropic lymphoma, which is a rare lymphoma characterized by the lymphoma cells confined in blood vessels and with a minimal involvement of lymph nodes, is presented. The clinical diagnosis was a malignant histiocytosis (histiocytic medullary reticulosis), since clinical manifestations were muscle weakness of the extremities, pancytopenia, fever and hepatosplenonephromegaly, and biopsy exhibited infiltration by histiocytic cells showing hemophagocytosis in the liver sinusoids and the bone marrow, the latter with myelofibrosis. Postmortem examination revealed lymphoma cell proliferation within small blood vessels of the kidneys, perineural tissue of extremities and lungs. Lymph nodes were not involved. In the liver, spleen and bone marrow, prominent infiltration of benign histiocytic cells with active hemophagocytosis was evident but there was no lymphoma cell infiltration. No viral infection, including Epstein-Barr (EBV), was demonstrable either serologically or immunohistochemically. Autopsy diagnosis was angiotropic lymphoma of B-cell origin (immunophenotypically CD 45+, CD 20-related+, CD 45R+, CD 45 RO-, CD 43-, CD 57-, CD 30-, CD 68-) accompanied by hemophagocytic syndrome clinicopathologically simulating malignant histiocytosis.