Abstract Details

Title Progressive Multifocal Leukoencephalopathy: A 25-Year Retrospective Cohort Study

Topic Infectious Disease

Presentation(s) S28 - Infectious Disease: Chronic Meningitis and the Immunosuppressed (2:36 PM-2:48 PM)

Poster/Presentation
Number 009

Objective To characterize the risk factors, clinical course, and treatment of progressive multifocal leukoencephalopathy (PML) patients diagnosed and followed over a 25-year epoch at Brigham and Women's Hospital and Massachusetts General Hospital.

Background Treatment options for PML are historically limited. However, recently, favorable outcomes have been reported in patients treated with immune checkpoint inhibitor therapy and infusion of virus-specific T-cells. With the new promise of effective therapy, understanding the diverse presentations of PML is increasingly important since early manifestations may be difficult to recognize but are most amenable to treatment.

Design/Methods Patients with a definite diagnosis of PML were identified by positive cerebrospinal fluid PCR for JC virus DNA or histopathology between 1 January 1994 and 1 January 2019. Demographic and PML-specific variables were recorded on symptomatic presentation and at follow up.

Results

There were 91 patients (n=53, 58% male) with confirmed PML with a median age at symptomatic onset of 53 years. HIV infection was the most common risk factor, identified in 49% (n=45). Other frequent risk factors included lymphoma, leukemia, or myelodysplasia, identified in 31% of patients (n=28); exposure to chemotherapeutic medications (30%, n=27); and exposure to monoclonal antibody therapies (19%, n=17). Thirty percent of the cohort was alive at the time of censoring, with a median mRS of 2 points, indicating slight disability at last follow up. Median survival following PML diagnosis in HIV-infected patients was longer than in HIV-uninfected patients (1,992 vs. 101 days, p=0.024). Forty patients survived more than 1 year after PML symptom onset, of whom 24 were HIV-infected (60%). Thirteen patients, all HIV-infected, survived more than 10 years after PML symptom onset.

Conclusions We add to the limited literature on PML by reporting its epidemiology in a large, U.S. observational cohort. These parameters may be useful for future clinical trials that measure survival and clinical outcomes.

Authors/Disclosures
Pria Anand, MD (Boston University School of Medicine) PRESENTER	Dr. Anand has nothing to disclose.
Gladia C. Hotan (Department of Brain and Cognitive Sciences, MIT)	Ms. Hotan has received research support from Institute of High Performance Computing.
Andre Vogel (Massachusetts General Hospital)	No disclosure on file
Nagagopal Venna, MBBS, FAAN (Massachusetts General Hospital)	Dr. Venna has nothing to disclose.
Farrah J. Mateen, MD, PhD, FAAN (Northwestern University Department of Neurology)	Dr. Mateen has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Mateen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Mateen has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Dr. Mateen has received research support from Genentech. The institution of Dr. Mateen has received research support from Amgen. The institution of Dr. Mateen has received research support from TG Therapeutics. Dr. Mateen has received intellectual property interests from a discovery or technology relating to health care.

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