Intravascular large cell lymphoma: Clinicopathological and immunohistochemical description of a case

A 51-year-old female patient was admitted to the Neurological clinic because of motor seizures with myoclonus of the right hand and right side of the face. The results of initial brain CT scan, chest X rays, EEG ultrasonography of the great blood vessels and laboratory tests made in another hospital were unremarkable. Because of repeated partial seizures transient aphasic disturbances, urinary sphincter disturbances and periodic low-grade fever the patient was transferred to our hospital four months after the disease onset. Laboratory tests and NMR suggested a nonspecific disseminated viral encephalitis. After administration of Endoxan she was ambulatory for several weeks and then became increasingly exhausted confused, febrile, dyspneic, tachypneic and developed a shock status with hepatorenal insufficiency. She died after 7 months of disease duration. Postmortem examination revealed intravascular collections of large atypical lymphoid cells of B cell line. Blood vessels changed in this way were common in the brain and rare in other organs including skin, lungs, heart, liver spleen and digestive system. They were not found in the lymph nodes and bone marrow. A biopsy was not done because of absence of symptomatic and swollen tissues. However, correlation of clinical feature and postmortem findings shows that absence of clinical manifestations in an organ does not mean lack of microscopic pathological changes and biopsy should be done regardless of absence of clinical signs. This case shows that intravascular lymphoma may mimic vasculitis or disseminated nonspecific viral encephalitis.