Evidence map›Paper›PMID 41822477›Full record

ArticleFrontiers in immunology2026

Predictive factors for severity and poor treatment response in children with Evans syndrome: A retrospective cohort study.

Monia Ben Khaled, Marwa Ben Ayed, Zaid Zaroui, Ilhem Ben Fraj, Samia Rekaya, Najla Mekki, Yosra Chebbi, Aicha Ben Taieb, Amani Merdassi, Ikram Zaiter and 9 more

Abstract read
In one paragraph

Article in Frontiers in immunology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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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

0 citing papers in PubMed.

No citing paper in PubMed yet.

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

19 authors.

Monia Ben KhaledFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Marwa Ben AyedFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Zaid ZarouiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Ilhem Ben FrajFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Samia RekayaFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Najla MekkiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Yosra ChebbiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Aicha Ben TaiebFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Amani MerdassiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Ikram ZaiterFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Takwa LamouchiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Ridha KoukiPediatric Immuno-Hematology Department, Bone Marrow Transplantation Center of Tunis, Tunis, Tunisia.
Houda KaabiDepartment of Immunohematology, National Center of Blood Transfusion, Tunis, Tunisia.
Hamida SlamaDepartment of Immunohematology, National Center of Blood Transfusion, Tunis, Tunisia.
Wafa AchourFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Mohamed BejaouiFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Fethi MellouliFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Imen Ben-MustaphaFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Monia OuederniFaculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Evans syndrome (ES) is a rare disorder characterized by autoimmune cytopenias affecting multiple blood cell lineages. In children, management remains particularly challenging due to the absence of clear guidelines for acute treatment and escalation to second-line therapies. We conducted a retrospective, longitudinal study (2010-2024) including pediatric patients (<18 years) with ES to identify predictors of severe presentation, need for second-line therapy, and fatal outcome. Predictors were identified using Cox Regression. Fifty patients were included (sequential = 27, concomitant = 23), with a median age at diagnosis of 4.1 years (IQR:1.5-8.5). Secondary ES was observed in 41(82%) cases, among which 38 (76%) had IEI. Severe clinical presentation at diagnosis occurred in 50% of patients and was independently associated with age < 24months and hemoglobin level < 80g/L. Corticosteroid dependence was observed in 34 cases (68%), with second-line therapy required in 31 patients (62%, cumulative risk=88%). This was associated with the presence of hepatomegaly and abnormal IgM levels. At last follow-up, 38(76%) patients were in remission and 19(38%) had relapsed. Fatal outcome (8 patients) was associated with age < 24 months at diagnosis (p=0.04), family history of IEI (p=10

Indexed as

Anemia, Hemolytic, AutoimmuneThrombocytopeniaAdrenal Cortex HormonesChildChild, PreschoolFemaleHumansInfantLongitudinal StudiesMalePrognosisRetrospective StudiesSeverity of Illness IndexTreatment OutcomeAdrenal Cortex Hormonesautoimmune cytopeniasEvans syndromeimmune dysregulationimmunosuppressive therapypediatricspredictorsprimary immunodeficienciessurvival

Identifiers

PMID41822477
PMCPMC12975918

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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.