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Malignant Intravascular Lymphomatosis

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An exceedingly rare cause of stroke due to the proliferation of neoplastic cells of lymphoid origin within small blood vessels. Malignant cells bear the immunophenotype of B- or T-cell lineage. The affinity of tumour cells for capillary endothelium may be explained by lymphocyte receptors for endothelial membrane antigens [Baumann TP et al. 2000]. There is typically a progressive Dementia/Delirium developing over a few weeks with seizures and stroke like episodes

The clinical course is slowly progressive, and it can be misdiagnosed as Limbic encephalitis, Herpes simplex encephalitis, CJD, Syphilis. There may be an increased LDH but blood and CSF may be free of lymphoma cells. May be an increase in CSF protein and Lymphocytes. EEG may show slowing. CT/MRI can be normal or show lesions. CT abdomen/pelvis for staging and looking for other disease.

Median survival of IML patients is only 6 months and there may be delayed diagnosis. May be reasonably to cover with Aciclovir until diagnosis established. Once diagnosis confirmed then start Chemotherapy. e.g. CHOP