TITLE

Progressive multifocal leukoencephalopathy in individuals with minimal or occult immunosuppression

AUTHOR(S)
Gheuens, Sarah; Pierone, Gerald; Peeters, Patrick; Koralnik, Igor J.
PUB. DATE
March 2010
SOURCE
Journal of Neurology, Neurosurgery & Psychiatry;Mar2010, Vol. 81 Issue 3, p247
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Background Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease of the brain, caused by reactivation of the polyomavirus JC (JCV). PML has classically been described in individuals with profound cellular immunosuppression such as patients with AIDS, haematological malignancies, organ transplant recipients or those treated with immunosuppressive or immunomodulatory medications for autoimmune diseases. Methods and case reports The authors describe five HIV seronegative patients with minimal or occult immunosuppression who developed PML including two patients with alcoholic cirrhosis, one with untreated dermatomyositis and two with idiopathic CD4+ T cell lymphocytopenia. The authors performed a review of the literature to find similar cases. Results The authors found an additional 33 cases in the literature. Of a total of 38 cases, seven (18.4%) had hepatic cirrhosis, five (13.2%) had renal failure, including one with concomitant hepatic cirrhosis, two (5.2%) were pregnant women, two (5.2%) had concomitant dementia, one (2.6%) had dermatomyositis, and 22 (57.9%) had no specific underlying diagnosis. Among these 22, five (22.7%) had low CD4+ T cell counts (0.080e0.2943109/l) and were diagnosed as having idiopathic CD4+ lymphocytopenia, and one had a borderline CD4+ T cell count of 0.3083109/l. The outcome was fatal in 27/38 (71.1%) cases within 1.5e120 months (median 8 months) from onset of symptoms, and 3/4 cases who harboured JCV-specific T cells in their peripheral blood had inactive disease with stable neurological deficits after 6e26 months of follow-up. Discussion These results indicate that PML can occur in patients with minimal or occult immunosuppression, and one can revisit the generally accepted notion that profound cellular immunosuppression is a prerequisite for the development of PML.
ACCESSION #
48406923

 

Related Articles

  • Immunosuppressants/methotrexate.  // Reactions Weekly;8/18/2012, Issue 1415, p25 

    The article describes the case of an elderly woman who developed progressive multifocal leukoencephalopathy after taking immunosuppressants and methotrexate.

  • Polyomavirus JC in the Context of Immunosuppression: A Series of Adaptive, DNA Replication-Driven Recombination Events in the Development of Progressive Multifocal Leukoencephalopathy. Johnson, Edward M.; Wortman, Margaret J.; Dagdanova, Ayuna V.; Lundberg, Patric S.; Daniel, Dianne C. // Clinical & Developmental Immunology;2013, p1 

    Polyomavirus JC (JCV) is the etiological agent of progressive multifocal leukoencephalopathy (PML), a demyelinating infection of oligodendrocytes in the brain. PML, a frequently fatal opportunistic infection in AIDS, has also emerged as a consequence of treatment with several new...

  • Immunosuppression Increases JC Polyomavirus Large T Antigen DNA Load in the Brains of Patients Without Progressive Multifocal Leukoencephalopathy. Bayliss, Julianne; Karasoulos, Tanja; McLean, Catriona A. // Journal of Infectious Diseases;Jan2013, Vol. 207 Issue 1, p133 

    The relationship between latent JC polyomavirus (JCV) infection and progressive multifocal leukoencephalopathy (PML) remains unclear. In this study, JCV DNA was quantified by real-time polymerase chain reaction (qPCR) in brain and kidney tissue from patients without PML. Immunosuppressed...

  • Pathogenesis of Progressive Multifocal Leukoencephalopathy—Revisited. White, Martyn K.; Khalili, Kamel // Journal of Infectious Diseases;Mar2011, Vol. 203 Issue 5, p578 

    Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system that is rare even though the proven etiological agent of PML, the polyomavirus JC (JC virus), is ubiquitous within the human population. The common feature of PML cases appears to be...

  • JC viraemia in kidney transplant recipients: to act or not to act? Rao, N.; Schepetiuk, S.; Choudhry, M.; Juneja, R.; Passaris, G.; Higgins, G.; Barbara, J. // Clinical Kidney Journal;Oct2012, Vol. 5 Issue 5, p471 

    The article presents case studies related to John Cunningham virus (JCV) in kidney transplant patients. It states that the clinical decision-making in renal transplantation coincided with the use of immunosuppressive regimens. It says that the JC polyomavirus cause progressive multifocal...

  • In Vitro and In Vivo Models for the Study of Human Polyomavirus Infection. Barth, Heidi; Solis, Morgane; Kack-Kack, Wallys; Soulier, Eric; Velay, Aurélie; Fafi-Kremer, Samira // Viruses (1999-4915);Oct2016, Vol. 8 Issue 10, p1 

    Developments of genome amplification techniques have rapidly expanded the family of human polyomaviruses (PyV). Following infection early in life, PyV persist in their hosts and are generally of no clinical consequence. High-level replication of PyV can occur in patients under immunosuppressive...

  • Progressive multifocal leukoencephalopathy - a rare but serious disease.  // Australian Prescriber;Feb2013, Vol. 36 Issue 1, p24 

    The article presents information regarding progressive multifocal leukoencephalopathy (PML), a demyelinating disease of the central nervous system. It mentions that PML is caused by a human polyomavirus, the JC virus. It also provides information regarding the risk factors, diagnosis and...

  • Human polyomavirus JC reactivation and pathogenetic mechanisms of progressive multifocal leukoencephalopathy and cancer in the era of monoclonal antibody therapies. Bellizzi, A.; Nardis, C.; Anzivino, E.; Rod�o, D.; Fioriti, D.; Mischitelli, M.; Chiarini, F.; Pietropaolo, V. // Journal of NeuroVirology;Feb2012, Vol. 18 Issue 1, p1 

    Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of the central nervous system caused by the neurotropic human polyomavirus JC (JCV) lytic infection of oligodendrocytes. PML was first described as a complication of lymphoproliferative disorders more than 50 years ago...

  • Progressive Multifocal Leukoencephalopathy and Newer Biological Agents. Berger, J. R. // Drug Safety;2010, Vol. 33 Issue 11, p969 

    Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the brain due to a polyoma virus, JC virus. Despite the ubiquity of this virus, PML is rare and almost always seen in association with an underlying immunosuppressive condition. In the last 30 years, AIDS has...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sign out of this library

Other Topics