Evidence map›Paper›PMID 30552536›Full record

Observational studyJournal of clinical immunology2019

Progressive Multifocal Leukoencephalopathy in Primary Immunodeficiencies.

Jérôme Hadjadj, Aurélien Guffroy, Christophe Delavaud, Guillaume Taieb, Isabelle Meyts, Anne Fresard, Nathalie Streichenberger, Anne-Sophie L'Honneur, Flore Rozenberg, Maud D'Aveni and 8 more

Abstract readObservational Study
PubMed Publisher
In one paragraph

Observational study in Journal of clinical immunology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.

0numbers the graph read from it
0cells of the map it votes in
19citing papers in PubMed
1.2field-weighted citation impact, top 17% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

19 citing papers in PubMed, 26 citations in OpenAlex.

  1. Article
  2. Current infectious disease management challenges in inborn errors of immunity.Annals of clinical microbiology and antimicrobials · 2025
    Review
  3. JC Polyomavirus Infection: A Narrative Review.Infectious diseases and therapy · 2025
    Review
  4. Review
  5. Inherited human RelB deficiency impairs innate and adaptive immunity to infection.Proceedings of the National Academy of Sciences of the United States of America · 2024
    Article
  6. Article
  7. Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Article
  13. Article
  14. Review
  15. An Update on XMEN Disease.Journal of clinical immunology · 2020
    Review
  16. Article
  17. Article
  18. Article
  19. Progressive Multifocal Leukoencephalopathy: Current Insights.Degenerative neurological and neuromuscular disease · 2019
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

18 authors at 11 institutions in 2 countries.

Jérôme HadjadjDepartment of Haematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, France et Université Paris Descartes, 149 rue de Sèvres, 75015, Paris, France.
Aurélien GuffroyDepartment of Clinical Immunology and Internal Medicine, National Reference Center for Autoimmune diseases, Hôpitaux Universitaires de Strasbourg, Strasbourg, France.
Christophe DelavaudDepartment of Adult Radiology, Necker Hospital, Paris, France.
Guillaume TaiebDepartment of Neurology, CHU Montpellier, Hôpital Guy de Chauliac, Montpellier, France.
Isabelle MeytsDepartment of Microbiology and Immunology, Childhood Immunology, KU Leuven, Leuven, Belgium.
Anne FresardDepartment of Infectious Diseases, CHU Saint-Etienne, Saint-Etienne, France.
Nathalie StreichenbergerDepartment of Pathology - Université Claude Bernard Lyon1 - Institut NeuroMyogène CNRS UMR 5310 - INSERM U1217, Hospices Civils de Lyon, Lyon, France.
Anne-Sophie L'HonneurDepartment of Virology, Université Paris Descartes et Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Paris, France.
Flore RozenbergDepartment of Virology, Université Paris Descartes et Assistance Publique-Hôpitaux de Paris, Hôpital Cochin, Paris, France.
Maud D'AveniDepartment of Hematology, University Hospital, Nancy, France.
Claire AguilarSorbonne Paris Cité, Necker Pasteur Center for Infectious Diseases and Tropical Medicine, APHP-Hôpital Necker-Enfants Malades, IHU Imagine, Université Paris Descartes, Paris, France.
Jérémie RosainStudy Center for Primary Immunodeficiencies, Necker-Enfants Malades Hospital, Assistance Publique Hôpitaux de Paris (APHP), Necker Medical School, Paris, France.
Capucine PicardStudy Center for Primary Immunodeficiencies, Necker-Enfants Malades Hospital, Assistance Publique Hôpitaux de Paris (APHP), Necker Medical School, Paris, France.
Nizar MahlaouiCentre de Référence Déficits Immunitaires Héréditaires (CEREDIH), Hôpital Universitaire Necker-Enfants Malades, Paris Descartes University, Paris, France.
Marc LecuitSorbonne Paris Cité, Necker Pasteur Center for Infectious Diseases and Tropical Medicine, APHP-Hôpital Necker-Enfants Malades, IHU Imagine, Université Paris Descartes, Paris, France.
Olivier HermineDepartment of Haematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, France et Université Paris Descartes, 149 rue de Sèvres, 75015, Paris, France.
Olivier LortholarySorbonne Paris Cité, Necker Pasteur Center for Infectious Diseases and Tropical Medicine, APHP-Hôpital Necker-Enfants Malades, IHU Imagine, Université Paris Descartes, Paris, France.
Felipe SuarezDepartment of Haematology, Assistance Publique-Hôpitaux de Paris, Hôpital Necker-Enfants Malades, France et Université Paris Descartes, 149 rue de Sèvres, 75015, Paris, France. felipe.suarez@aphp.fr.
Hôpital Necker-Enfants Malades · FRAssistance Publique – Hôpitaux de Paris · FRUniversité Paris Cité · FRCentre de Référence Déficits Immunitaires Héréditaires · FRCentre Hospitalier Régional et Universitaire de Nancy · FRCentre Hospitalier Universitaire de Saint-Étienne · FRDepartment of Virology · FRHôpital Gui de Chauliac · FRHôpitaux Universitaires de Strasbourg · FRInserm · FRKU Leuven · BE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

purposeProgressive multifocal leukoencephalopathy (PML) is a rare but severe demyelinating disease caused by the polyomavirus JC (JCV) in immunocompromised patients. We report a series of patients with primary immune deficiencies (PIDs) who developed PML.

methodsRetrospective observational study including PID patients with PML. Clinical, immunological, imaging features, and outcome are provided for each patient.

resultsEleven unrelated patients with PIDs developed PML. PIDs were characterized by a wide range of syndromic or genetically defined defects, mostly with combined B and T cell impairment. Genetic diagnosis was made in 7 patients. Before the development of PML, 10 patients had recurrent infections, 7 had autoimmune and/or inflammatory manifestations, and 3 had a history of malignancies. Immunologic investigations showed CD4

conclusionPML is a rare but fatal complication of PIDs. Many cases are secondary to immunosuppressive therapy warranting careful evaluation before initiation subsequent immunosuppression during PIDs.

Indexed as

AdolescentAdultAzathioprineB-LymphocytesFemaleHumansImmunologic Deficiency SyndromesImmunotherapyJC VirusLeukoencephalopathy, Progressive MultifocalLymphopeniaMaleMiddle AgedRetrospective StudiesRituximabT-LymphocytesAzathioprineRituximabTumor Necrosis Factor-alphacombined immunodeficienciesimmunosuppressive therapypolyomavirus JCprimary immunodeficienciesProgressive multifocal leukoencephalopathy

Identifiers

PMID30552536
OpenAlexW2905441239

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.