Early management of hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a systematic literature review
Bibliographic record
Abstract
Objectives: A systematic literature review was undertaken to summarise and analyse the available evidence on the initial hemophagocytic lymphohistiocytosis and macrophage activation syndrome (HLH/MAS) treatment according to the distinctive pathologic background and/or triggering factors. Methods: An electronic search in the main medical databases (PubMed, EMBASE, and Cochrane Library) was conducted, in order to select the studies providing information related to the HLH/MAS therapy. To further maximise the evidence derived from this systematic review, we focused our attention on clinical studies that included and described >10 HLH/MAS cases. Results: This literature search led to identify 18,020 articles. After the screening by title, removal of duplicated records, and full-text assessment of the relevant articles, the final search output consisted of 167 articles that provided enough information on the medical management of patients with HLH/MAS In total, 77 clinical studies (including >10 patients) describing the early treatment of HLH/MAS have been included in the present analysis. Conclusions: Primary HLH is typically managed according to the HLH-94 or HLH-2004 protocols, with the main aim of bringing the patient to a hematopoietic stem cell transplant (HSCT). Secondary forms of HLH/MAS can arise in 3 main clinical contexts, namely rheumatic disorders, oncological diseases, and previously healthy patients developing specific and/or severe infections. Accordingly, different and variable therapeutic approaches have been applied to these 3 categories of secondary HLH/MAS. Despite the survival improvements achieved in the last decades in patients with HLH/MAS, there is a strong need for prospective and controlled studies focused on each specific group of HLH/MAS.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.006 | 0.026 |
| Meta-epidemiology (narrow) | 0.002 | 0.001 |
| Meta-epidemiology (broad) | 0.007 | 0.006 |
| Bibliometrics | 0.014 | 0.010 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.002 | 0.001 |
| Insufficient payload (model declined to judge) | 0.005 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".