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Long-term prognosis and overall mortality in patients with progressive multifocal leukoencephalopathy
Jinnam Kim1,2,3, Changhyup Kim1,2, Jung Ah Lee1,2
1Division of Infectious Diseases, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, 50-1 Yonsei-Ro, Seodaemun-Gu, Seoul, 03722, Korea.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a rare but fatal opportunistic infection and mainly occurs in patients with immunosuppressive conditions. Despite the increasing number of patients receiving immunosuppressive treatments, studies on PML are still lacking due to its low prevalence and incidence. We retrospectively reviewed patients diagnosed with PML in two tertiary hospitals in South Korea from 1999 to 2021. Total of 47 PML patients were included. Of 27 patients (57.4%) were diagnosed with human immunodeficiency virus (HIV). Median last follow-up modified Rankin Scale (mRS) score was higher in the non-HIV PML group than that in the HIV group (5 vs. 4, p = 0.020). Median survival duration was lower in the non-HIV group (184 vs. 1,564 days). The 1-year and overall mortality rates of PML patients were significantly higher in the non-HIV group than that in HIV group (60.0% vs. 25.9%, p = 0.019; 80.0% vs. 40.7%, p = 0.007). Initial mRS score (HR 1.685, p = 0.038) and highly active antiretroviral therapy (HAART) in HIV patients (HR 0.374, p = 0.013) had a significant effect on overall mortality. Our findings suggest that early detection of PML with low mRS score and early initiation of HAART in patients with HIV may improve prognosis.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare infection. Early detection and treatment, especially highly active antiretroviral therapy (HAART) for human immunodeficiency virus (HIV) patients, can improve survival rates.
Area of Science:
- Neurology
- Infectious Diseases
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal opportunistic infection.
- PML primarily affects individuals with compromised immune systems, often due to immunosuppressive treatments.
- Despite increased use of immunosuppressants, research on PML remains limited due to its low incidence.
Purpose of the Study:
- To investigate the characteristics and outcomes of PML patients in South Korea.
- To compare prognosis and survival rates between PML patients with and without human immunodeficiency virus (HIV).
- To identify factors influencing mortality in PML.
Main Methods:
- Retrospective review of 47 PML patients diagnosed between 1999 and 2021 in two South Korean tertiary hospitals.
- Analysis of patient demographics, clinical data, and treatment outcomes.
- Comparison of outcomes between HIV-positive and HIV-negative PML cohorts.
Main Results:
- Of 47 patients, 27 (57.4%) had HIV. Non-HIV PML patients had poorer outcomes, including higher modified Rankin Scale (mRS) scores at follow-up (5 vs. 4) and shorter median survival (184 vs. 1,564 days).
- Mortality rates were significantly higher in the non-HIV group: 1-year (60.0% vs. 25.9%) and overall (80.0% vs. 40.7%).
- Initial mRS score and highly active antiretroviral therapy (HAART) in HIV patients were significant predictors of overall mortality.
Conclusions:
- Early detection of PML with a low mRS score is crucial.
- Prompt initiation of HAART in HIV-positive PML patients is associated with improved survival.
- Further research is needed to optimize management strategies for PML across different patient populations.
