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Progressive multifocal leukoencephalopathy in a patient with glioblastoma.

ABSTRACT: Malignant gliomas are aggressive malignancies which inevitably recur despite multimodality treatment. In a subset of patients who are longer term survivors of this disease, progressive radiologic worsening can also occur from late effects of radiation rather than recurrent tumor, a differential diagnosis that is commonly considered in this setting. However, other causes for radiologic progression are not as well recognized and could potentially confound management leading to incorrect treatment decisions. Progressive multifocal leukoencephalopathy (PML) is a rare infectious demyelinating disease of the central nervous system seen primarily in immunocompromised patients, the early diagnosis and treatment of which remains a challenge. Here, we report a case of a long term survivor with glioblastoma whose diagnostic and therapeutic management was confounded by the development of PML. We review the radiological features and clinical course of this patient to highlight the dramatic neurological course in the setting of a highly malignant tumor, and emphasize the unusual changes in diffusion weighted images, and the need for clinical suspicion for early diagnosis of PML.

SUBMITTER: Wu J

PROVIDER: S-EPMC5546753 | biostudies-other | 2011 Jul

REPOSITORIES: biostudies-other

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Project description:Progressive multifocal leukoencephalopathy (PML) usually occurs in patients with severe immunosuppression, hematological malignancies, chronic inflammatory conditions or receiving organ transplant. Recently, PML has also been observed in patients treated with monoclonal antibodies. By taking advantage of the availability of samples from a multiple sclerosis (MS) patient treated with natalizumab, the antibody anti-?4 integrin, who developed PML and was monitored starting before therapy initiation, we investigated the fate of T and B lymphocytes in the onset of PML. Real-time PCR was used to measure new T- and B-cell production by means of T-cell receptor excision circle (TREC) and K-deleting recombination excision circle (KREC) analysis and to quantify transcripts for CD34, terminal-deoxynucleotidyltransferase, and V pre-B lymphocyte gene 1. T- and B-cell subsets and T-cell heterogeneity were measured by flow cytometry and spectratyping. The data were compared to those of untreated and natalizumab-treated MS patients and healthy donors. Before therapy, a patient who developed PML had a low TREC and KREC number; TRECs remained low, while KRECs and pre-B lymphocyte gene 1 transcripts peaked at 6 months of therapy and then decreased at PML diagnosis. Flow cytometry confirmed the deficient number of newly produced T lymphocytes, counterbalanced by an increase in TEMRA cells. The percentage of naive B cells increased by approximately 70% after 6 months of therapy, but B lymphocyte number remained low for the entire treatment period. T-cell heterogeneity and immunoglobulins were reduced. Although performed in a single patient, all results showed that an immune deficit, together with an increase in newly produced B cells a few months after therapy initiation, may predispose the patient to PML. These findings indicate the TREC/KREC assay is a potential tool to identify patients at risk of developing PML and may provide insights into the immunological involvement of monoclonal antibody-associated therapies.

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Progressive multifocal leukoencephalopathy in a patient with glioblastoma.

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