Evidence map›Paper›PMID 42170321›Full record

ArticleJournal of human immunity2026

Lymphoproliferation in inborn errors of immunity: From challenging diagnosis to histologic revision.

Mattia Moratti, Beatrice Rivalta, Antonello Cardoni, Veronica Santilli, Enrico Attardi, Emma Concetta Manno, Riccardo Ciudino, Silvia Di Cesare, Cristina Cifaldi, Chiara Mengoli and 20 more

Abstract read
In one paragraph

Article in Journal of human immunity, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
–field-weighted citation impact
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

5 citing papers in PubMed.

  1. Observational
  2. Article
  3. Article
  4. Article
  5. Article
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

30 authors.

Mattia MorattiDepartment of Biomedicine and Prevention, Molecular Medicine and Applied Biotechnology, University of Rome Tor Vergata, Rome, Italy.ORCID https://orcid.org/0000-0003-1133-9563
Beatrice RivaltaUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0003-2199-289X
Antonello CardoniPathology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.ORCID https://orcid.org/0000-0003-3468-0473
Veronica SantilliUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0003-1329-6827
Enrico AttardiDepartment of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy.ORCID https://orcid.org/0009-0008-4801-5048
Emma Concetta MannoUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0009-0009-0095-8167
Riccardo CiudinoHaematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0009-0006-7245-584X
Silvia Di CesareUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0002-9912-7997
Cristina CifaldiDepartment of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.ORCID https://orcid.org/0000-0002-7170-7279
Chiara MengoliPediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, University of Bologna, Bologna, Italy.ORCID https://orcid.org/0009-0001-7763-7451
Edoardo MuratorePediatric Hematology and Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0001-6954-3140
Samuele NaviglioDepartment of Pediatrics, Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste, Italy.ORCID https://orcid.org/0000-0002-1915-9965
Paola SelvaLaboratory of Immuno-Haematology-Laboratorio Unico Metropolitano, Azienda USL, Bologna, Italy.ORCID https://orcid.org/0009-0007-0198-5234
Simona FerrariMedical Genetics Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0002-4087-1535
Gigliola Di MatteoDepartment of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.ORCID https://orcid.org/0000-0001-8344-5635
Alessandro BroccoliIRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli," Bologna, Italy.ORCID https://orcid.org/0000-0001-5633-7313
Nicola CotugnoUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0002-7748-1581
Donato AmodioUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0003-4550-3018
Riccardo MasettiPediatric Hematology and Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0002-1264-057X
Elena FacchiniPediatric Hematology and Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0009-0003-4044-9216
Pier Luigi ZinzaniIRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seràgnoli," Bologna, Italy.ORCID https://orcid.org/0000-0002-2112-2651
Marcello LanariPediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, University of Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0002-2586-314X
Cinzia MilitoDepartment of Molecular Medicine, "Sapienza" University of Rome, Rome, Italy.ORCID https://orcid.org/0000-0003-2581-9250
Alberto TommasiniDepartment of Pediatrics, Institute for Maternal and Child Health IRCCS Burlo Garofolo, Trieste, Italy.ORCID https://orcid.org/0000-0002-6943-7927
Rita De VitoPathology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.ORCID https://orcid.org/0000-0002-7450-8398
Andrea FinocchiUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0003-0958-8536
Rita AlaggioPathology Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.ORCID https://orcid.org/0000-0003-3915-3816
Elena SabattiniHaematopathology Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0002-4136-3591
Caterina CancriniUnit of Clinical Immunology and Vaccinology, IRCCS Bambino Gesù Children Hospital, Rome, Italy.ORCID https://orcid.org/0000-0001-8410-9617
Francesca ContiPediatric Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, University of Bologna, Bologna, Italy.ORCID https://orcid.org/0000-0002-3665-8926

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Inborn errors of immunity (IEI) are genetic disorders that not only heighten infection risk but also disrupt immune regulation, frequently leading to lymphoid tissue overgrowth known as lymphoid proliferations (LPD). We retrospectively reviewed 38 patients with genetically or clinically confirmed IEI and persistent LPD, comparing those with nonneoplastic/reactive hyperplasia to those who developed overt lymphoid neoplasm (lymphoma). Overall, 26% developed lymphoma-predominantly classical Hodgkin lymphoma or diffuse large B cell lymphoma-often after earlier IEI onset. Immunophenotyping and principal component analysis revealed that patients with common variable immunodeficiency developing Hodgkin lymphoma shared a distinctive T cell profile, differing from immunocompetent lymphoma cases. Centralized histologic re-evaluation reclassified several presumed lymphoma as nonneoplastic/reactive hyperplasia and identified Castleman-like and germinal center transformation patterns in nonneoplastic/reactive LPD. Notably, elevated blood IgM and circulating T follicular helper cells mirrored IgM deposits and PD-1

Identifiers

PMID42170321
PMCPMC13177383

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Registered trials

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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.