INTRAVASCULAR LYMPHOMATOSIS OF THE CNS - CLINICOPATHOLOGICAL STUDY AND SEARCH FOR EXPRESSION OF ONCOPROTEINS AND EPSTEIN-BARR-VIRUS

Five cases of intravascular lymphomatosis (IVL) are reported. Diffuse or focal cerebral signs suggestive of vascular disease occurred in fou r cases. but case 5 presented with symptoms similar to Creutzfeld-Jako b disease. Clinical course ranged from two to eight months and diagnos is was made in all cases by autopsy. Neoplastic lymphoid cells mainly lodged in lumina of small vessels in many organs, but infarction was c onfined to the CNS. Some extravascular tumor cells were regularly seen . All cases corresponded to high-grade Non-Hodgkin lymphomas of B-cell type and displayed high proliferation indices. Different from finding s in primary cerebral and nodal lymphomas, neither p53 nor bcl-2 oncop roteins were detectable. Absence of EBV genome and EBV latent membrane protein from IVL was demonstrated for the first time.