title: Progressive multifocal leukoencephalopathy with concurrent Richter's syndrome.
abstract: 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.
authors.affiliation: Department of Leukemia, at the University of Texas M.D. Anderson Cancer Center Houston, 77030, USA.
authors.forename: E D
authors.initials: ED
authors.lastname: Saad
authors.forename: D A
authors.initials: DA
authors.lastname: Thomas
authors.forename: S
authors.initials: S
authors.lastname: O'Brien
authors.forename: G N
authors.initials: GN
authors.lastname: Fuller
authors.forename: L J
authors.initials: LJ
authors.lastname: Medeiros
authors.forename: A
authors.initials: A
authors.lastname: Forman
authors.forename: M
authors.initials: M
authors.lastname: Albitar
authors.forename: D
authors.initials: D
authors.lastname: Schomer
authors.forename: H M
authors.initials: HM
authors.lastname: Kantarjian
authors.forename: M J
authors.initials: MJ
authors.lastname: Keating
mesh_terms: 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