CoChat
Sign Up
Cited by 282Autoimmune and Inflammatory Disorders ResearchKawasaki Disease and Coronary ComplicationsInflammasome and immune disorders

Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis

Eloseily, Esraa M., Weiser, Peter, Crayne, Courtney B., Haines, Hilary, Mannion, Melissa L., Stoll, Matthew L., Beukelman, Timothy, Atkinson, T. Prescott +1 more·2020·Arthritis & Rheumatology
DOI 10.1002/art.41103
Open this paper in CoChat — free account

Abstract

Objective: To assess the benefit of the recombinant human interleukin‐1 receptor antagonist anakinra in treating pediatric patients with secondary hemophagocytic lymphohistiocytosis ( HLH )/macrophage activation syndrome ( MAS ) associated with rheumatic and nonrheumatic conditions. Methods: A retrospective chart review of all anakinra‐treated patients with secondary HLH / MAS was performed at Children's of Alabama from January 2008 through December 2016. Demographic, clinical, laboratory, and genetic characteristics, outcomes data, and information on concurrent treatments were collected from the records and analyzed using appropriate univariate statistical approaches to assess changes following treatment and associations between patient variables and outcomes. Results: Forty‐four patients with secondary HLH / MAS being treated with anakinra were identified in the electronic medical records. The median duration of hospitalization was 15 days. The mean pretreatment serum ferritin level was 33,316 ng/ml and dropped to 14,435 ng/ml (57% decrease) within 15 days of the start of anakinra treatment. The overall mortality rate in the cohort was 27%. Earlier initiation of anakinra (within 5 days of hospitalization) was associated with reduced mortality ( P = 0.046), whereas thrombocytopenia (platelet count <100,000/μl) and STXBP 2 mutations were both associated with increased mortality ( P = 0.008 and P = 0.012, respectively). In considering patients according to their underlying diagnosis, those with systemic juvenile idiopathic arthritis ( JIA ) had the lowest mortality rate, with no deaths among the 13 systemic JIA patients included in the study ( P = 0.006). In contrast, those with an underlying hematologic malignancy had the highest mortality rate, at 100% (n = 3). Conclusion: These findings suggest that anakinra appears to be effective in treating pediatric patients with non–malignancy‐associated secondary HLH / MAS , especially when it is given early in the disease course and when administered to patients who have an underlying rheumatic disease.

Integrity signals

Registry

Crossref · 10.1002/art.41103

Cites

50 of 50 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

  • The interleukin-1-receptor antagonist anakinra is effective as a single agent and in combination with other agents for the treatment of some forms of cytokine storm.

    “The interleukin-1–receptor antagonist anakinra is effective as a single agent and in combination with other agents for the treatment of some forms of cytokine storm.”

    From Cytokine storm (2020) · cites this paper as [33]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

Related

Authors
Eloseily, E. M., Weiser, P., Crayne, C. B., Haines, H., Mannion, M. L., Stoll, M. L., Beukelman, T., Atkinson, T. P., & Cron, R. Q.
Affiliations
University of Alabama at Birmingham (US)
Corresponding author
Cron, Randy Q.
ORCID
Venue
Arthritis & Rheumatology
Publisher
Wiley
Published
2019-12-26
Volume
72
Issue
2
Pages
326-334
ISSN
  • 2326-5191 (ISSN-L)
  • 2326-5205
DOI
10.1002/art.41103
OpenAlex
W2973124663
Cited by
282
References
50
Citations by year
2026: 19 · 2025: 25 · 2024: 36 · 2023: 43 · 2022: 53 · 2021: 45 · 2020: 56 · 2019: 2 · 2015: 2 · 2013: 1
FWCI
27.53
Type
journal article
Language
en
Open access
closed
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Arthritis & Rheumatology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
  • Health Sciences › Medicine › Surgery › Kawasaki Disease and Coronary Complications
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Inflammasome and immune disorders
Keywords
anakinra, secondary hemophagocytic lymphohistiocytosis, macrophage activation syndrome, pediatric patients, interleukin-1 receptor antagonist, systemic juvenile idiopathic arthritis, thrombocytopenia, serum ferritin, mortality, hematologic malignancies, rheumatic diseases
MeSH terms
Adolescent, Child, Child, Preschool, Female, Humans, Male, Retrospective Studies, Treatment Outcome, Lymphohistiocytosis, Hemophagocytic, Interleukin 1 Receptor Antagonist Protein, Macrophage Activation Syndrome

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review