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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
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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.
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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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