Progressive multifocal leukoencephalopathy with concurrent Richter's syndrome.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating infectious disease caused by the JC virus. It was originally described in patients with chronic lymphocytic leukemia (CLL). Richter's syndrome, or transformation to large cell Lymphoma, occurs in approximately 3% of patients with CLL, and carries a poor prognosis. We report a patient with documented PML and concurrent Richter's transformation outside the central nervous system. Before establishing a definitive diagnosis of PML, radiation therapy to the presumed lymphomatous brain lesion had been considered, raising the issue of whether stereotactic brain biopsy should be considered in every patient in a similar situation. Although this is likely a rare occurrence, patients with Richter's transformation documented at an extra-neural site and a brain lesion may benefit from the establishment of an infectious diagnosis which would influence therapy.
Saad
E D
ED
Department of Leukemia, at the University of Texas M.D. Anderson Cancer Center Houston, 77030, USA.
Thomas
D A
DA
O'Brien
S
S
Fuller
G N
GN
Medeiros
L J
LJ
Forman
A
A
Albitar
M
M
Schomer
D
D
Kantarjian
H M
HM
Keating
M J
MJ
D002471:Cell Transformation, Neoplastic; D006801:Humans; D007577:JC Virus / Q000302:isolation & purification; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications* / Q000473:pathology; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000473:pathology / Q000821:virology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000209:etiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged