[An autopsy case of intravascular malignant lymphomatosis with hemophagocytic syndrome, mental confusion and liver dysfunction].

A 66-year-old Japanese man was admitted to Hitachi General Hospital because of fatigue, fever, edema, and icterus. A blood examination showed anemia, leukocytosis, and hyperbilirubinemia. Antibiotics did not alleviate the inflammatory symptoms. On the 13th hospital day, the patient demonstrated mental confusion, with progressive anemia and thrombocytopenia. Bone marrow aspiration revealed an increase of macrophages showing erythrophagocytosis. A diagnosis of hemophagocytic syndrome was made, but no viral infection or hematological malignancy could be detected. The patient was treated with gamma-globulin and methylprednisolone for hemophagocytic syndrome, but died of respiratory insufficiency with progressive hyperbilirubinemia and trombocytopenia on the 29th hospital day, A postmortem examination showed proliferation of lymphoma cells within the small blood vessels of the brain, lungs, liver, and many other organs, but the bone marrow was not involved. An immunohistochemical examination resulted in a diagnosis of intravascular malignant lymphomatosis (IML) of B cell origin. No viral infection was detected with in situ hybridization methods. Although IML occasionally accompanies thrombocytopenia, infiltration of lymphoma cells in the bone marrow is rare. Lymphoma associated hemophagocytic syndrome may cause cytopenia in some patients with IML, as indicated in this case.