Evidence map›Paper›PMID 38627860›Full record

ReviewAdvances in rheumatology (London, England)2024

Hemophagocytic lymphohistiocytosis and macrophage activation syndrome: two rare sides of the same devastating coin.

Flavio Sztajnbok, Adriana Rodrigues Fonseca, Leonardo Rodrigues Campos, Kátia Lino, Marta Cristine Félix Rodrigues, Rodrigo Moulin Silva, Rozana Gasparello de Almeida, Sandro Félix Perazzio, Margarida de Fátima Fernandes Carvalho

Open access · diamondAbstract readReview
In one paragraph

Review in Advances in rheumatology (London, England), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed
15.0field-weighted citation impact, top 1% 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

25 citing papers in PubMed, 33 citations in OpenAlex.

  1. Article
  2. Nanomaterials Drive In Vivo CAR Immune Cells Engineering.Advanced materials (Deerfield Beach, Fla.) · 2026
    Review
  3. Review
  4. Article
  5. Article
  6. Article
  7. Article
  8. Article
  9. [Musculoskeletal Complications in Rheumatic Diseases].Journal of the Korean Society of Radiology · 2026
    Review
  10. Article
  11. Article
  12. Article
  13. Review
  14. Review
  15. Applicability of Hscore and Adapted HLH 2004 for Diagnosis of Adult HLH in Indian population-A Prospective Study.Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion · 2025
    Article
  16. [A rare differential diagnosis of fever of unknown origin].Innere Medizin (Heidelberg, Germany) · 2025
    Article
  17. Review
  18. Article
  19. Article
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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

9 authors at 5 institutions in 1 country.

Flavio SztajnbokDepartment of Pediatrics, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil. flaviosztajnbok@hotmail.com.ORCID http://orcid.org/0000-0002-7301-9664
Adriana Rodrigues FonsecaDepartment of Pediatrics, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
Leonardo Rodrigues CamposHospital Universitário Antônio Pedro, Universidade Federal Fluminense, Niterói, Brazil.
Kátia LinoHospital Universitário Antônio Pedro, Universidade Federal Fluminense, Niterói, Brazil.
Marta Cristine Félix RodriguesPediatric Rheumatology Division, Instituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
Rodrigo Moulin SilvaHospital Universitário Pedro Ernesto, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil.
Rozana Gasparello de AlmeidaInstituto de Puericultura e Pediatria Martagão Gesteira, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
Sandro Félix PerazzioRare Diseases Committee, Brazilian Society of Rheumatology (SBR), Rio de Janeiro, Brazil.
Margarida de Fátima Fernandes CarvalhoRare Diseases Committee, Brazilian Society of Rheumatology (SBR), Rio de Janeiro, Brazil.
Universidade Federal do Rio de Janeiro · BRUniversidade Federal Fluminense · BRUniversidade do Estado do Rio de Janeiro · BRUniversidade Estadual de Londrina · BRUniversidade Federal de São Paulo · BR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.

Indexed as

Autoimmune DiseasesLymphohistiocytosis, HemophagocyticMacrophage Activation SyndromeChildDiagnosis, DifferentialHumansCytokinesHemophagocytic lymphohistiocytosisJuvenile idiopathic arthritisMacrophage activation syndrome

Identifiers

PMID38627860
PMCPMC13488743
OpenAlexW4394855578

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.