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Published on: October 19, 2014
[Progressive multifocal leukoencephalopathy (PML) in chronic lymphatic leukemia (CLL). Review of the literature and
C Holzapfel1, C Kellinghaus, R Lüttmann
1Klinik und Poliklinik für Neurologie, Universitätsklinikum Münster, Albert-Schweitzer-Strasse 33, 48129 Münster.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is an infectious disease of the central nervous system caused by the JC virus. Progressive multifocal leukoencephalopathy represents a reactivation of the JC virus after long-standing immunosuppression. Also, PML plays an important role as an opportunistic infection in patients with AIDS. The average time of survival in patients with PML in combination with chronic lymphatic leukemia (CLL) (n = 17 in the literature) is 4.3 months, and therapeutic options are not established. We report the case of a patient with CLL and PML. Clinical symptoms are slight hemiparesis of the right side, mainly appearing as a disturbance of motor function. In MRI, a typical subcortical lesion was shown, and JC virus DNA was positive in the CSF by PCR. Because of first positive results in treatment of PML in patients with AIDS, therapy with cidofovir was started. After treatment for 16 months, symptoms are stable, the PML-induced lesions in MRI are in regression, and JC virus DNA is not detectable in the CSF.
Insights
Progressive multifocal leukoencephalopathy (PML), a JC virus infection, can occur with chronic lymphatic leukemia (CLL). Cidofovir treatment stabilized symptoms and improved MRI lesions in a CLL patient with PML.
Area of Science:
- Neuroscience
- Infectious Diseases
- Oncology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, fatal demyelinating disease of the central nervous system caused by the JC virus (JCV).
- PML is often associated with immunosuppression, particularly in patients with advanced HIV/AIDS and hematologic malignancies like chronic lymphatic leukemia (CLL).
- Therapeutic options for PML, especially in non-HIV patients, are limited, with a poor prognosis often reported in the literature.
Observation:
- This report details a case of a patient diagnosed with both CLL and PML.
- The patient presented with mild right-sided hemiparesis and motor function disturbances.
- Cerebrospinal fluid (CSF) analysis revealed JC virus DNA positivity via PCR, and MRI showed characteristic subcortical lesions.
Findings:
- The patient received cidofovir therapy, an antiviral agent showing initial promise in PML cases associated with AIDS.
- Following 16 months of treatment, the patient's clinical symptoms remained stable.
- Significant regression of PML-induced MRI lesions was observed, and JC virus DNA became undetectable in the CSF.
Implications:
- This case suggests that cidofovir may be a viable therapeutic option for PML in patients with CLL.
- The findings indicate potential for improved outcomes and disease control in this challenging patient population.
- Further research into antiviral therapies for PML in immunocompromised individuals beyond HIV is warranted.
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