Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Feb 22;6(2):ofz024.
doi: 10.1093/ofid/ofz024. eCollection 2019 Feb.

The Incidence and Predisposing Factors of John Cunningham Virus-Induced Progressive Multifocal Leukoencephalopathy in Southern Finland: A Population-Based Study

Marge Kartau  1 , Auli Verkkoniemi-Ahola  1 , Anders Paetau  2 , Maarit Palomäki  3 , Rita Janes  4 , Matti Ristola  5 , Maija Lappalainen  6 , Veli-Jukka Anttila  5
Affiliations

The Incidence and Predisposing Factors of John Cunningham Virus-Induced Progressive Multifocal Leukoencephalopathy in Southern Finland: A Population-Based Study

Marge Kartau et al. Open Forum Infect Dis. .

Abstract

Background: The aim of this study was to assess the prevalence, incidence rate (IR), predisposing factors, survival rate, and diagnostic delay of progressive multifocal leukoencephalopathy (PML) across medical specialties. Another objective was to survey how PML diagnosis was made in the studied cases.

Methods: This is a cross-sectional retrospective observational study of PML cases across different medical specialties during 2004-2016 in the Finnish Capital Region and Southern Finland. Data were obtained from clinical records, clinical microbiology, pathology and radiology department records, and human immunodeficiency virus (HIV) quality register medical records.

Results: A total of 31 patients were diagnosed with PML. The prevalence of PML was 1.56 per 100 000 people and the IR was 0.12 per 100 000 individuals per year during 2004-2016. Hematologic malignancies (n = 19) and HIV/acquired immune deficiency syndrome (n = 5) were the most common underlying diseases, and all patients who had malignant diseases had received cancer treatment. Before PML diagnosis, 21 (67.7%) patients were treated with chemotherapy, 14 (45.2%) patients with rituximab, and 1 patient (3.2%) with natalizumab. Two patients (6.5%) had no obvious immunocompromising disease or treatment. Neither gender, age, first symptoms, previous medication, nor underlying disease influenced the survival of PML patients significantly. The 5-year survival rate was poor, at less than 10%.

Conclusions: The majority of PML patients in our study had a predisposing disease or had immunosuppressive or monoclonal antibody therapy. In the future, broader use of immunosuppressive and immunomodulatory medications may increase incidence of PML among patients with diseases unassociated with PML. Safety screening protocols for John Cunningham virus and PML are important to prevent new PML cases.

Keywords: HIV; JC virus; monoclonal antibodies; progressive multifocal leukoencephalopathy; rituximab.

PubMed Disclaimer

Figures

Figure 1.
Figure 1.
Annual cases of progressive multifocal leukoencephalopathy (PML).
See this image and copyright information in PMC

References

    1. Assetta B, Maginnis MS, Gracia Ahufinger I, et al. . 5-HT2 receptors facilitate JC polyomavirus entry. J Virol 2013; 87:13490–8. - PMC - PubMed
    1. Gheuens S, Wüthrich C, Koralnik IJ. Progressive multifocal leukoencephalopathy: why gray and white matter. Annu Rev Pathol 2013; 8:189–215. - PubMed
    1. Eng PM, Turnbull BR, Cook SF, et al. . Characteristics and antecedents of progressive multifocal leukoencephalopathy in an insured population. Neurology 2006; 67:884–6. - PubMed
    1. Iacobaeus E, Burkill S, Bahmanyar S, et al. . The national incidence of PML in Sweden, 1988–2013. Neurology 2018; 90:e498–e506. - PMC - PubMed
    1. Palazzo E, Yahia SA. Progressive multifocal leukoencephalopathy in autoimmune diseases. Joint Bone Spine 2012; 79:351–5. - PubMed