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Progressive multifocal leukoencephalopathy without overt immunosuppression
Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports a...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Lippincott Williams & Wilkins
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10545270/ https://www.ncbi.nlm.nih.gov/pubmed/37773871 http://dx.doi.org/10.1097/MD.0000000000035265 |
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author | Jain, Varun Branstetter, Hannah Savaram, Srikar Vasquez, Matthew Swords, Gabriel Aghili-Mehrizi, Sina Rees, John Rivera-Zengotita, Marie Montalvo, Mayra Chuquilin, Miguel Patterson, Addie Rempe, Torge |
author_facet | Jain, Varun Branstetter, Hannah Savaram, Srikar Vasquez, Matthew Swords, Gabriel Aghili-Mehrizi, Sina Rees, John Rivera-Zengotita, Marie Montalvo, Mayra Chuquilin, Miguel Patterson, Addie Rempe, Torge |
author_sort | Jain, Varun |
collection | PubMed |
description | Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports and series. Its prevalence, overall frequency, and prognosis are largely unknown. This is a single-center retrospective review of all University of Florida cases with the ICD10 PML diagnosis code (A81.2). PML without overt immunosuppression was defined as absence of human immunodeficiency virus (HIV) infection, hematological malignancy, immunomodulatory/-suppressive medications, autoimmune conditions with a propensity for PML (sarcoidosis, systemic lupus erythematosus). Cases that did not fulfill criteria for clinically or histologically definite PML were excluded. Of 52 patients with the ICD10 code A 81.2, 17 fulfilled definite diagnostic criteria for PML. Overt immunosuppression was identified in 15/17 (88.2%) cases (10/17 (58.8%): human immunodeficiency virus; 5/17 (29.4%): immunomodulatory/-suppressive medication). Two/seventeen (11.8%) cases were consistent with PML without overt immunosuppression. Possible contributing factors were a preceding dog bite and mild hypogammaglobulinemia M (39 mg/dL) in case 1 and significant alcohol use without evidence for liver disease in case 2. Both cases were fatal within 6 (case 1) and 2 (case 2) months. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression if clinical and radiographic findings are suggestive of the diagnosis. |
format | Online Article Text |
id | pubmed-10545270 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Lippincott Williams & Wilkins |
record_format | MEDLINE/PubMed |
spelling | pubmed-105452702023-10-03 Progressive multifocal leukoencephalopathy without overt immunosuppression Jain, Varun Branstetter, Hannah Savaram, Srikar Vasquez, Matthew Swords, Gabriel Aghili-Mehrizi, Sina Rees, John Rivera-Zengotita, Marie Montalvo, Mayra Chuquilin, Miguel Patterson, Addie Rempe, Torge Medicine (Baltimore) 5300 Progressive multifocal leukoencephalopathy (PML) is a central nervous system disease caused by the human polyomavirus 2 that usually occurs in a setting of immunodeficiency. PML without overt immunosuppression is considered a rare occurrence but has been described in multiple previous case reports and series. Its prevalence, overall frequency, and prognosis are largely unknown. This is a single-center retrospective review of all University of Florida cases with the ICD10 PML diagnosis code (A81.2). PML without overt immunosuppression was defined as absence of human immunodeficiency virus (HIV) infection, hematological malignancy, immunomodulatory/-suppressive medications, autoimmune conditions with a propensity for PML (sarcoidosis, systemic lupus erythematosus). Cases that did not fulfill criteria for clinically or histologically definite PML were excluded. Of 52 patients with the ICD10 code A 81.2, 17 fulfilled definite diagnostic criteria for PML. Overt immunosuppression was identified in 15/17 (88.2%) cases (10/17 (58.8%): human immunodeficiency virus; 5/17 (29.4%): immunomodulatory/-suppressive medication). Two/seventeen (11.8%) cases were consistent with PML without overt immunosuppression. Possible contributing factors were a preceding dog bite and mild hypogammaglobulinemia M (39 mg/dL) in case 1 and significant alcohol use without evidence for liver disease in case 2. Both cases were fatal within 6 (case 1) and 2 (case 2) months. The results suggest that PML without overt immunosuppression may be more common than previously described. Therefore, PML should be considered even in the absence of overt immunosuppression if clinical and radiographic findings are suggestive of the diagnosis. Lippincott Williams & Wilkins 2023-09-29 /pmc/articles/PMC10545270/ /pubmed/37773871 http://dx.doi.org/10.1097/MD.0000000000035265 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by-nc/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial License 4.0 (CCBY-NC) (https://creativecommons.org/licenses/by-nc/4.0/) , where it is permissible to download, share, remix, transform, and buildup the work provided it is properly cited. The work cannot be used commercially without permission from the journal. |
spellingShingle | 5300 Jain, Varun Branstetter, Hannah Savaram, Srikar Vasquez, Matthew Swords, Gabriel Aghili-Mehrizi, Sina Rees, John Rivera-Zengotita, Marie Montalvo, Mayra Chuquilin, Miguel Patterson, Addie Rempe, Torge Progressive multifocal leukoencephalopathy without overt immunosuppression |
title | Progressive multifocal leukoencephalopathy without overt immunosuppression |
title_full | Progressive multifocal leukoencephalopathy without overt immunosuppression |
title_fullStr | Progressive multifocal leukoencephalopathy without overt immunosuppression |
title_full_unstemmed | Progressive multifocal leukoencephalopathy without overt immunosuppression |
title_short | Progressive multifocal leukoencephalopathy without overt immunosuppression |
title_sort | progressive multifocal leukoencephalopathy without overt immunosuppression |
topic | 5300 |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10545270/ https://www.ncbi.nlm.nih.gov/pubmed/37773871 http://dx.doi.org/10.1097/MD.0000000000035265 |
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