Progressive Multifocal Leukoencephalopathy (PML)

Treatment

Definitive diagnosis (laboratory): evidence of JCV-DNA in CSF AND presence of compatible clinical-radiological picture

Definitive diagnosis (histology): typical histological findings with in situ evidence of JCV-DNA antigen or JCV-DNA AND presence of compatible clinical-radiological picture

Presumptive diagnosis: compatible clinical-radiological picture if JCV-DNA in CSF negative or not performed. JCV-DNA in plasma may complement PML diagnosis, particularly if CSF not available. May also be a marker of disease progression [8]

Person off-ART Initiate ART immediately (following general guidelines for treatment, see Initial Combination Regimen for ART-naïve Adult PLWH), INSTI may reasonably be preferred, given the importance of rapid immune reconstituion in PML. Attention should be made to development of IRIS, see IRIS
Person on-ART, HIV-VL failure Optimise ART (following general guidelines for treatment, see Virological Failure), INSTI may reasonably be preferred, given the importance of rapid immune reconstituion in PML. Attention should be made to development of IRIS, see IRIS
Person on-ART, treated for weeks-months
or on effective cART
Continue current ART


Note:
There is no specific treatment for JCV infection that proved to be effective in PML outside of anecdotal case reports, therefore there is no recommendation to use the following drugs which previously or occasionally were used in PML: Alpha-IFN, cidofovir, corticosteroids (except for treatment of IRIS-PML, see IRIS), cytarabine, iv immunoglobulins, mefloquine, mirtazapine. Newer immune-based approaches have shown some efficacy, including Interleukin-7, infusion of polyomavirus-specific HLA-matched T-cells, anti-PD1 inhibitors, but no conclusive data are currently supporting their recommendation for clinical use