Progressive multifocal leukoencephalopathy treated with interleukin-7
Background: Progressive multifocal leukoencephalopathy (PML) is a rare devastating brain infection caused by the JC polyomavirus (JCPyV) in patients with impaired cellular immune responses. In the absence of effective anti-viral therapy, it is unclear if the use of interleukin-7 (IL-7) could enhance...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-10-01
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| Series: | Clinical Infection in Practice |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2590170220300364 |
| _version_ | 1818664474235633664 |
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| author | Karl B. Alstadhaug Christine Hanssen Rinaldo Liv Osnes Irini Sereti Hilde K. Ofte |
| author_facet | Karl B. Alstadhaug Christine Hanssen Rinaldo Liv Osnes Irini Sereti Hilde K. Ofte |
| author_sort | Karl B. Alstadhaug |
| collection | DOAJ |
| description | Background: Progressive multifocal leukoencephalopathy (PML) is a rare devastating brain infection caused by the JC polyomavirus (JCPyV) in patients with impaired cellular immune responses. In the absence of effective anti-viral therapy, it is unclear if the use of interleukin-7 (IL-7) could enhance cellular immune responses required to survive PML. Case report: A 78-year-old woman who had been treated with corticosteroids for pulmonary sarcoidosis 25 years earlier developed PML associated with severe idiopathic T-cell lymphopenia characterized by expression of programmed cell death-1 (PD-1) receptors on circulating CD4+ and CD8+ T-cells. The patient was diagnosed 12 weeks after her initial symptoms, and treatment with mirtazapine was started immediately. The disease progressed, however, and treatment with IL-7 was initiated 17 weeks after initial symptomatology begun. Results: At week 21, clinical improvement was observed and despite persistent JCPyV positive cerebrospinal fluid until week 36, the patient continued to improve remarkably, both clinically and radiologically, along with increasing peripheral lymphocytes and declining PD-1 expression. Treatment was well tolerated without late complications. More than one year after treatment (week 71) the patient cared for herself, and had no signs of PML recurrence, relapse of sarcoidosis, or cancer. A mild increase, however, in serum angiotensin-converting enzyme and plasma soluble interleukin-2 receptor was observed. Conclusion: Faced with PML and lymphopenia, in the absence of conventional therapies to reverse the underlying immunodeficiency, IL-7 therapy may be helpful in combatting the JC polyomavirus. |
| first_indexed | 2024-12-17T05:33:19Z |
| format | Article |
| id | doaj.art-26e01492f8664e5dac94981cc39562f4 |
| institution | Directory Open Access Journal |
| issn | 2590-1702 |
| language | English |
| last_indexed | 2024-12-17T05:33:19Z |
| publishDate | 2020-10-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Clinical Infection in Practice |
| spelling | doaj.art-26e01492f8664e5dac94981cc39562f42022-12-21T22:01:41ZengElsevierClinical Infection in Practice2590-17022020-10-017100049Progressive multifocal leukoencephalopathy treated with interleukin-7Karl B. Alstadhaug0Christine Hanssen Rinaldo1Liv Osnes2Irini Sereti3Hilde K. Ofte4Department of Neurology, Nordland Hospital Trust, Bodø 8092, Norway; Institute of Clinical Medicine, University of Tromsø, Norway; Corresponding author at: Department of Neurology, Nordland Hospital Trust, Bodø 8092, Norway.Department of Microbiology and Infection Control, University Hospital of North, Norway; Metabolic and Renal Research Group, Department of Clinical Medicine, UiT The Arctic University of Norway, NorwayDepartment of Immunology, Oslo University Hospital, NorwayNational Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health, Bethesda, MD, USADepartment of Neurology, Nordland Hospital Trust, Bodø 8092, NorwayBackground: Progressive multifocal leukoencephalopathy (PML) is a rare devastating brain infection caused by the JC polyomavirus (JCPyV) in patients with impaired cellular immune responses. In the absence of effective anti-viral therapy, it is unclear if the use of interleukin-7 (IL-7) could enhance cellular immune responses required to survive PML. Case report: A 78-year-old woman who had been treated with corticosteroids for pulmonary sarcoidosis 25 years earlier developed PML associated with severe idiopathic T-cell lymphopenia characterized by expression of programmed cell death-1 (PD-1) receptors on circulating CD4+ and CD8+ T-cells. The patient was diagnosed 12 weeks after her initial symptoms, and treatment with mirtazapine was started immediately. The disease progressed, however, and treatment with IL-7 was initiated 17 weeks after initial symptomatology begun. Results: At week 21, clinical improvement was observed and despite persistent JCPyV positive cerebrospinal fluid until week 36, the patient continued to improve remarkably, both clinically and radiologically, along with increasing peripheral lymphocytes and declining PD-1 expression. Treatment was well tolerated without late complications. More than one year after treatment (week 71) the patient cared for herself, and had no signs of PML recurrence, relapse of sarcoidosis, or cancer. A mild increase, however, in serum angiotensin-converting enzyme and plasma soluble interleukin-2 receptor was observed. Conclusion: Faced with PML and lymphopenia, in the absence of conventional therapies to reverse the underlying immunodeficiency, IL-7 therapy may be helpful in combatting the JC polyomavirus.http://www.sciencedirect.com/science/article/pii/S2590170220300364JC virusJCPyVIRISImmunodeficiencyIdiopathic T-cell lymphocytopeniaInterleukin-7 |
| spellingShingle | Karl B. Alstadhaug Christine Hanssen Rinaldo Liv Osnes Irini Sereti Hilde K. Ofte Progressive multifocal leukoencephalopathy treated with interleukin-7 Clinical Infection in Practice JC virus JCPyV IRIS Immunodeficiency Idiopathic T-cell lymphocytopenia Interleukin-7 |
| title | Progressive multifocal leukoencephalopathy treated with interleukin-7 |
| title_full | Progressive multifocal leukoencephalopathy treated with interleukin-7 |
| title_fullStr | Progressive multifocal leukoencephalopathy treated with interleukin-7 |
| title_full_unstemmed | Progressive multifocal leukoencephalopathy treated with interleukin-7 |
| title_short | Progressive multifocal leukoencephalopathy treated with interleukin-7 |
| title_sort | progressive multifocal leukoencephalopathy treated with interleukin 7 |
| topic | JC virus JCPyV IRIS Immunodeficiency Idiopathic T-cell lymphocytopenia Interleukin-7 |
| url | http://www.sciencedirect.com/science/article/pii/S2590170220300364 |
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