Intravascular lymphomatosis (of B- or T-cell origin) is a rare lymphoprolif
erative disorder characterised by neoplastic proliferation of lymphoid cell
s within the lumen of capillaries, small veins and arteries with no or mini
mal involvement of the parenchyma. Its predilection sites are the skin and
the brain. We studied a 44-year-old man who presented with a 2 year history
of unexplained LDH elevation followed by a neurological syndrome without s
ystemic involvement. Brain biopsy showed an intravascular lymphoma of the B
-cell lineage. This report illustrates the diagnostic challenge of this rar
e disorder with a grim prognosis. (C) 1999 Elsevier Science B.V. All rights
reserved.