Disease response criteria in Langerhans cell histiocytosis: a global view
Bibliographic record
Abstract
Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasm with heterogeneous presentations. The discovery of BRAFV600E and other MAPK pathway mutations drastically transformed the treatment landscape, especially for high-risk LCH and CNS-LCH. While treatment strategies for children and adults are somewhat similar, response assessment methodologies remain highly dichotomized.Currently, separate treatment response criteria exist for children and adults, especially in therapeutic trials. Considering the rapid evolution of targeted MAPK-inhibitor therapies, along with ultrasensitive detection of minimal residual disease biomarkers (e.g., circulating BRAFV600E-encoding DNA) and sophisticated imaging tools (18F-FDG-PET and whole-body MRI), harmonization of response criteria in LCH is clearly warranted. The Histiocyte Society Global LCH Treatment Response Harmonization Task Force, a collaborative network of pediatric and adult LCH experts, is set to propose updated pediatric LCH treatment response criteria, which will also serve as the foundation for a universal response assessment tool for pediatric and adult LCH. In this review, we focus on the past, present, and likely future of response assessment in LCH patients, and discuss needs that remain unmet in the targeted therapy era.
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How this classification was reachedexpand
Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.002 | 0.001 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.000 |
| Insufficient payload (model declined to judge) | 0.000 | 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 teacher head, 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".