Evidence map›Paper›PMID 38633254›Full record

ArticleFrontiers in immunology2024

Case report: EBV-related eye orbits and sinuses lymphohistiocytic infiltration responsive to rituximab in a patient with X lymphoproliferative syndrome type 1.

Giuliana Giardino, Vittoria Lanni, Massimo Mascolo, Daniela Russo, Emilia Cirillo, Roberta Romano, Francesca Cillo, Laura Grilli, Maria Rosaria Prencipe, Adriana Iuliano and 6 more

Open access · goldAbstract readCase Reports
PubMed Full text at PMC Publisher
In one paragraph

Article in Frontiers in immunology, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.5field-weighted citation impact, top 38% 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

2 citing papers in PubMed, 1 citations in OpenAlex.

  1. Article
  2. 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

16 authors at 3 institutions in 1 country.

Giuliana GiardinoDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Vittoria LanniDepartment of Neurosciences, Reproductive Sciences and Dentistry, University of Naples Federico II, Naples, Italy.
Massimo MascoloDepartment of Advanced Biomedical Sciences, Pathology Unit, Federico II University of Naples, Naples, Italy.
Daniela RussoDepartment of Advanced Biomedical Sciences, Pathology Unit, Federico II University of Naples, Naples, Italy.
Emilia CirilloDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Roberta RomanoDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Francesca CilloDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Laura GrilliDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Maria Rosaria PrencipeDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Adriana IulianoDepartment of Neurosciences, Reproductive Sciences and Dentistry, University of Naples Federico II, Naples, Italy.
Giovanni UccelloDepartment of Neurosciences, Reproductive Sciences and Dentistry, University of Naples Federico II, Naples, Italy.
Carmela De FuscoPediatric Hematology and Oncology, Pausilipon Hospital, Naples, Italy.
Giuseppe MennaPediatric Hematology and Oncology, Pausilipon Hospital, Naples, Italy.
Giulia ScaliaClinical and Experimental Cytometry Unit, Centre for Advanced Biotechnology Franco Salvatore, CEINGE, Naples, Italy.
Giuseppe PortellaDepartment of Translational Medical Sciences, Virology Section, Federico II University of Naples, Naples, Italy.
Claudio PignataDepartment of Translational Medical Sciences, Pediatric Section, Federico II University of Naples, Naples, Italy.
Federico II University Hospital · ITUniversity of Naples Federico II · ITSantobono Children's Hospital · IT

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background and aims: X lymphoproliferative syndrome type 1 (XLP1) is a rare inborn error of immunity due to mutations of Methods: We report the case of a child affected with XLP1 who presented with an incomplete HLH, triggered by Epstein-Barr virus (EBV) and treated with rituximab, involving orbits and paranasal sinuses. Results: The lesion was indistinguishable from lymphoma, complicating diagnosis and treatment. In addition, considering the high incidence of lymphoma in patients with XLP1, histology helped define its nature, driving therapeutic choices. Conclusion: We described an unusual presentation of incomplete HLH in a patient affected with XLP1: an EBV-driven infiltration of the orbits and paranasal sinuses. This led us to a challenging differential diagnosis of lymphoma-associated hemophagocytic syndrome, which can be frequently observed in patients with XLP1. Considering the extremely poor prognosis of this clinical finding, we sought for a prompt diagnosis and managed to obtain it and to immediately establish the right treatment on the basis of the pathological finding.

Indexed as

Epstein-Barr Virus InfectionsImmunologic Deficiency SyndromesLymphohistiocytosis, HemophagocyticLymphomaLymphoproliferative DisordersChildHerpesvirus 4, HumanHumansRituximabRituximabEBVeye lymphohistiocytic infiltrationrituximabSH2D1AX lymphoproliferative syndrome type 1

Identifiers

PMID38633254
PMCPMC11021630
OpenAlexW4393900476

What OpenQuestion holds

Textmetadata
LicenceCC BY
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.