Carson KR, Evens AM, Richey EA et al. Blood 2009;113:4834–40.
Progressive multifocal leukoencephalopathy (PML) is a progressive, usually fatal disease caused by JC polyoma virus (JCV) that has been associated with the use of rituximab. This research group assembled 57 cases of rituximab-associated PML in order to gather information on risk factors and outcome.
JC polyoma virus (JCV) infection is endemic, affecting >90% of adults. In the presence of immunocompromise – such as advanced HIV, intensive chemotherapy, or congenital immuno-deficiency – reactivation of the infection can occur, resulting in a demyelinating disease of the central nervous system (CNS) that is usually fatal. Progressive multifocal leukoencephalopathy (PML) has now been associated with the use of two novel immunomodulatory drugs: natalizumab, an antibody directed against α4 integrin and used in the treatment of multiple sclerosis, and rituximab, an antibody against CD20 that is used to treat hematological malignancies and autoimmune diseases.