Refractory T-cell anergy and rapidly fatal progressive multifocal leukoencephalopathy after prolonged CTLA4 therapy

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Abstract

Progressive multifocal leukoencephalopathy (PML) is a deadly demyelinating disease due to central nervous system replication of the human polyomavirus JC virus (JCV) in immunosuppressed patients. The only effective therapeutic approach is to restore anti-JCV T-cell responses. In this study, we describe a case of rapidly fatal PML with JCV T-cell anergy in a renal transplant patient treated with CTLA4-Ig (belatacept, a CD28-B7 costimulation blocker and T-cell anergy inducer). T-cell anergy could not be reversed despite several therapeutic approaches. Progressive multifocal leukoencephalopathy secondary to biotherapy- induced T-cell anergy may thus represent a subset of PML with major resistance to anti-JCV immune recovery.

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Dekeyser, M., de Herve, M. G. de G., Hendel-Chavez, H., Labeyrie, C., Adams, D., Nasser, G. A., … Taoufik, Y. (2017). Refractory T-cell anergy and rapidly fatal progressive multifocal leukoencephalopathy after prolonged CTLA4 therapy. Open Forum Infectious Diseases, 4(2). https://doi.org/10.1093/ofid/ofx100

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