Intravascular malignant Lymphomatosis presenting as demyelinating disease

Abstract

Background & Significance : Intravascular lymphomatosis is a systemic, rare, malignant B- or T-cell lymphoma with remarkable affinity for the endothelial cells of small vessels, particularly in the skin and central nervous system. Most cases are not diagnosed until postmortem because of the marked variability in clinical presentation, ranging from acute focal deficits to a gradually progressive dementia. Cytologic diagnosis is made difficult by the frequent absence of lymphomatous cells in the serum and cerebrospinal fluid. It is characterized by neoplastic proliferation of lymphoid cells within the lumen of small veins and arteries with minimal involvement of the parenchyma. Case : We experienced a 41 year-old woman who present dysarthria. At first, we diagnosed as ADEM after check of brain MRI. In CBC & electrolyte, no specific finding was seen. After steroid therapy, she was improved & discharged. But 1 month after, she presented with seizure and fever. We diagnosed her as Multiple sclerosis and treated again by Steroid. In spite of steroid therapy, she was worsen and expired after 31 days treatment. After her death, we have done autopsy of her brain. In pathologic finding, many lymphocytes were seen in all lumina of arterioles and small arteries. And subsequent immunocytochemical identification of CD20 and CD 45 showed and an atypical large B-cell population within the same lumina of vessels. In intravascular space, lymphocytes stained by B-cell marker(Leu20) were aggregated and scattered outside blood vessels. In T-cell maker strain(UCHL1), there were only a few marked cells. Conclusions or Commentes : We describe a 41-year-old woman with unusual clinical features and radiologic pictures resembling demyelinating disease, who was diagnosed in postmortem as intravascular lymphomatosis.