ANGIOTROPIC B-CELL LYMPHOMA WITH HEMOPHAGOCYTIC SYNDROME

A unique autopsy case of angiotropic lymphoma, which is a rare lymphom a characterized by the lymphoma cells confined in blood vessels and wi th a minimal involvement of lymph nodes, is presented. The clinical di agnosis was a malignant histiocytosis (histiocytic medullary reticulos is), since clinical manifestations were muscle weakness of th e extrem ities, pancytopenia, fever and hepatosplenonephromegaly, and biopsy ex hibited 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 s mall blood vessels of the kidneys, perineural tissue of extremities an d 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 infiltrat ion. No viral infection, including Epstein-Barr (EBV), was demonstrabl e either serologically or immunohistochemically. Autopsy diagnosis was angiotropic lymphoma of B-cell origin (immunophenotypically CD 45+, C D 20-related +, CD 45R +, CD 45 RO -, CD 43 -, CD 57 -, CD 30 -, CD 68 -) accompanied by hemophagocytic syndrome clinicopathologically simul ating malignant histiocytosis.