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The natural history of progressive multifocal leukoencephalopathy in patients with AIDS. Canadian PML Study Group
Abstract:
Progressive multifocal leukoencephalopathy (PML) is usually a fatal neurological disease. The natural history of PML in patients with human immunodeficiency virus infection was analyzed. The correlations between CD4+ lymphocyte count, previous diagnosis of AIDS, treatment with cytarabine, and survival time are reported for 28 individuals for whom the diagnosis of PML was confirmed by histopathologic examination. For 16 patients (57%), PML was the AIDS-defining illness. For these 16 patients, the mean (+/- SD) survival time after presentation was 7.5 +/- 7.6 months (range, 1-31 months), whereas that for the 12 patients (43%) for whom AIDS was previously diagnosed was 3.2 +/- 2.8 months (range, 1-11 months) (P = .01). The overall mean (+/- SD) CD4+ cell count was 85 +/- 82/mm3 (range, 12-349/mm3). The mean (+/- SD) survival time for patients with CD4+ cell counts of > or = 90/mm3 at the time of presentation was 9.4 +/- 8.7 months, while that for patients with CD4+ cell counts of < 90/mm3 at the time of presentation was 3.6 +/- 1.8 months (P = .03). The nine patients did not benefit from treatment with cytarabine.
Insights
Progressive multifocal leukoencephalopathy (PML) in HIV patients is often fatal. Higher CD4 counts and PML as an initial AIDS diagnosis correlate with longer survival, while cytarabine treatment showed no benefit.
Area of Science:
- Neurology
- Infectious Diseases
- Immunology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease.
- PML frequently affects individuals with compromised immune systems, particularly those with human immunodeficiency virus (HIV) infection.
- Understanding the natural history and prognostic factors of PML in HIV-infected individuals is crucial for patient management.
Purpose of the Study:
- To analyze the natural history of PML in HIV-infected patients.
- To investigate correlations between CD4+ lymphocyte count, prior AIDS diagnosis, cytarabine treatment, and survival time.
- To report findings for 28 histopathologically confirmed PML cases.
Main Methods:
- Retrospective analysis of 28 HIV-infected patients with histopathologically confirmed PML.
- Data collection included CD4+ lymphocyte counts, AIDS diagnosis status, cytarabine treatment, and survival duration.
- Statistical analysis to determine correlations between variables and survival.
Main Results:
- PML was the AIDS-defining illness in 57% of patients.
- Patients diagnosed with PML as the AIDS-defining illness had a mean survival of 7.5 months vs. 3.2 months for those with a prior AIDS diagnosis (P = .01).
- Higher CD4+ counts (≥90/mm³) at presentation correlated with longer survival (9.4 months vs. 3.6 months; P = .03). Cytarabine treatment did not improve survival.
Conclusions:
- In HIV-infected individuals, PML diagnosis as the initial AIDS-defining illness and higher CD4+ counts are associated with improved survival.
- Cytarabine is not an effective treatment for PML in this patient population.
- These findings highlight the importance of immune status in PML prognosis and underscore the need for effective therapeutic strategies.