Refractory T-Cell Anergy and Rapidly Fatal Progressive Multifocal Leukoencephalopathy After Prolonged CTLA4 Therapy

• Published in: Open forum infectious diseases Vol. 4; no. 2; p. ofx100
• Main Authors: Dekeyser, Manon, de Goër de Herve, Marie-Ghislaine, Hendel-Chavez, Houria, Labeyrie, Céline, Adams, David, Nasser, Ghaïdaa Adebs, Gasnault, Jacques, Durrbach, Antoine, Taoufik, Yassine
• Format: Journal Article
• Language: English
• Published: United States Oxford University Press 01-03-2017
• Subjects: Brief Report; JCV-specific T cells; progressive multifocal leukoencephalopathy; transplantation
• ISSN: 2328-8957; 2328-8957
• DOI: 10.1093/ofid/ofx100

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.