title: Progressive multifocal leukoencephalopathy: what's new?
abstract: Progressive multifocal leukoencephalopathy (PML), a severe demyelinating disease that is caused by human JC polyomavirus, was first described as a complication of immune suppression 50 years ago and emerged as a major complication of HIV infection in the 1980s. The prognosis has remained dismal since then, with discouraging results from clinical trials of various therapeutic approaches, including immunomodulation and/or inhibition of viral replication. PML is caused by reactivation of latent JC virus, and serotonergic 5-HT(2a) receptors have been identified as being critical for viral infection of glial cells. In recent years, immunosuppressive therapeutic antibodies have been associated with an increased incidence rate of PML. Here, the authors review findings on the pathogenesis of PML and the encouraging case reports of novel treatments.
authors.affiliation: Department of Oncology, Transplants and Advances in Medicine, Division of Hematology, University of Pisa, Pisa, Italy. dfocosi@tin.it
authors.forename: Daniele
authors.initials: D
authors.lastname: Focosi
authors.forename: Tuccori
authors.initials: T
authors.lastname: Marco
authors.forename: Richard Eric
authors.initials: RE
authors.lastname: Kast
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Maggi
authors.forename: Luca
authors.initials: L
authors.lastname: Ceccherini-Nelli
authors.forename: Mario
authors.initials: M
authors.lastname: Petrini
mesh_terms: D006801:Humans; D016867:Immunocompromised Host / Q000276:immunology*; D007165:Immunosuppression Therapy / Q000009:adverse effects* / Q000379:methods*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000188:drug therapy / Q000503:physiopathology* / Q000628:therapy* / Q000821:virology