Diagnosis and treatment of intravascular lymphomatosis

Objective: To describe a patient with unusually good outcome of a rare, hig h-grade lymphoma that often involves the nervous system. Design: Case report. Setting: University hospital. Case: A 70-year-old pharmacist first presented with meningoencephalitis lik e symptoms and 4 months later with acute confusional state followed by comp lex partial status epilepticus. Diagnosis of intravascular lymphomatosis wa s made using detection and biopsy of a bilateral adrenal tumor. Main Outcome and Results: Polychemotherapy consisting of CHOP (cyclophospha mide, doxorubicin hydrochloride, vincristine sulfate, and prednisone) led t o complete remission. The patient's survival time currently exceeds 2 1/2 y ears. Conclusions: The possibility of intravascular lymphomatosis should he consi dered in adult patients with unclear meningoencephalitic syndrome, acute co nfusional stale, dementia, or other unexplained neurologic conditions with signs of a systemic disease. In intravascular lymphomatosis. as in other hi gh-grade non-Hodgkin lymphomas, CHOP polychemotherapy should be the standar d treatment.